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Kondo H, Ogura K, Kawai A. The impact of adjuvant therapy in extraskeletal osteosarcoma: A propensity score-matched analysis of 226 cases from the bone and Soft Tissue Tumor Registry in Japan. EUROPEAN JOURNAL OF SURGICAL ONCOLOGY 2025; 51:110118. [PMID: 40347593 DOI: 10.1016/j.ejso.2025.110118] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/12/2025] [Revised: 04/14/2025] [Accepted: 05/01/2025] [Indexed: 05/14/2025]
Abstract
BACKGROUND Extraskeletal osteosarcoma (ESOS) is a rare malignant soft tissue tumor for which optimal treatment strategies remain unclear. Although several retrospective studies have suggested the potential efficacy of adjuvant therapies, selection bias has limited the reliability of these findings. This study analyzes the largest ESOS cohort using the Japanese Bone and Soft Tissue Tumor (BSTT) Registry, employing propensity score matching to evaluate treatment outcomes while minimizing selection bias. METHODS We retrospectively analyzed 226 ESOS cases from the BSTT Registry from 2006 to 2022. Patient characteristics, treatment modalities, and survival outcomes were evaluated using Kaplan-Meier analysis and Cox proportional hazards modeling. Propensity score matching was performed to assess the efficacy of adjuvant therapies while controlling for patient background factors. RESULTS The 5-year overall survival rate was 53.6 %. In the unmatched cohort, tumor location (p = 0.012), tumor size (p = 0.014), and surgical treatment (p < 0.001) significantly influenced survival. After propensity score matching, adjuvant chemotherapy demonstrated a significant survival benefit (p = 0.044), while adjuvant radiotherapy showed no significant improvement (p = 0.951). No significant difference was observed between osteosarcoma-type and soft tissue sarcoma-type chemotherapy regimens (p = 0.213 after matching). CONCLUSIONS This large cohort study, utilizing propensity score matching, provides evidence for the efficacy of adjuvant chemotherapy in ESOS treatment. While surgical resection remains the cornerstone of treatment, our findings support incorporating adjuvant chemotherapy into treatment strategies, regardless of regimen type. Prospective studies are needed to confirm these findings.
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Affiliation(s)
- Hiroya Kondo
- Department of Musculoskeletal Oncology and Rehabilitation Medicine, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo 104-0045, Japan
| | - Koichi Ogura
- Department of Musculoskeletal Oncology and Rehabilitation Medicine, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo 104-0045, Japan.
| | - Akira Kawai
- Department of Musculoskeletal Oncology and Rehabilitation Medicine, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo 104-0045, Japan
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Komatsu A, Higashi Y, Lin CK, Chen YP, Wu SH, Suzuki M, Matsumoto K, Tamanoi F. Accumulation of Small-Size, Highly Dispersive Mesoporous Silica Nanoparticles in a Tumor in Both Chorioallantoic Membrane and Mouse Models. Cells 2025; 14:734. [PMID: 40422237 DOI: 10.3390/cells14100734] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/25/2025] [Revised: 05/09/2025] [Accepted: 05/15/2025] [Indexed: 05/28/2025] Open
Abstract
(1) Background: The chorioallantoic membrane (CAM) model has the potential to contribute to the development of personalized medicine based on individual cancer patients. We previously established the CAM model using patient-derived CIC-DUX4 sarcoma cells. We also used the CAM model for characterization and a comparison with the mouse model by examining the tumor accumulation of small-size, highly dispersive mesoporous silica nanoparticles (MSNs). (2) Method: In this study, we transplanted a variety of cancer cell lines, including patient-derived osteosarcoma (OS) and extraskeletal osteosarcoma (ESOS) cells. Patient-derived OS, ESOS and other cell lines were transplanted onto CAMs. The proliferation of cancer cells within CAM tumors was confirmed using H&E staining. For the comparison of the CAM and mouse models, rhodamine B-labeled MSNs were administered intravenously to CAMs and to xenograft mice. Tumor accumulation was evaluated by examining fluorescence and by confocal microscopy. The biodistribution of MSNs was examined by measuring the Si content by ICP. (3) Results: H&E staining demonstrated the proliferation of cancer cells of OS, ESOS and others on CAMs. While growth patterns and morphologies varied among different cancer types, H&E staining confirmed the establishment of tumors. As for the tumor accumulation, both the CAM and mouse models showed that MSNs were selectively accumulated in the tumors in both the CAM and mouse models. (4) Conclusions: We have expanded the range of CAM models by using a variety of cancer cells, including patient-derived cell lines. We also report that the small-size, highly dispersive MSNs exhibit excellent tumor accumulation in both the CAM and mouse models. These results point to the usefulness of the CAM model for patient-derived cancer cells as well as for evaluating drug carriers for tumor targeting.
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Affiliation(s)
- Aoi Komatsu
- Institute for Integrated Cell-Material Sciences, Institute for Advanced Study, Kyoto University, Kyoto 606-8501, Japan
| | - Yuya Higashi
- Institute for Integrated Cell-Material Sciences, Institute for Advanced Study, Kyoto University, Kyoto 606-8501, Japan
| | - Cong-Kai Lin
- Graduate Institute of Nanomedicine and Medical Engineering, Taipei Medical University, Taipei 11031, Taiwan
- International Ph.D. Program in Biomedical Engineering, Taipei Medical University, Taipei 11031, Taiwan
| | - Yi-Ping Chen
- Graduate Institute of Nanomedicine and Medical Engineering, Taipei Medical University, Taipei 11031, Taiwan
- International Ph.D. Program in Biomedical Engineering, Taipei Medical University, Taipei 11031, Taiwan
| | - Si-Han Wu
- Graduate Institute of Nanomedicine and Medical Engineering, Taipei Medical University, Taipei 11031, Taiwan
- International Ph.D. Program in Biomedical Engineering, Taipei Medical University, Taipei 11031, Taiwan
| | - Minoru Suzuki
- Institute for Integrated Radiation and Nuclear Science, Kyoto University, Osaka 590-0494, Japan
| | - Kotaro Matsumoto
- Institute for Integrated Cell-Material Sciences, Institute for Advanced Study, Kyoto University, Kyoto 606-8501, Japan
| | - Fuyuhiko Tamanoi
- Institute for Integrated Cell-Material Sciences, Institute for Advanced Study, Kyoto University, Kyoto 606-8501, Japan
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Liu H, Tian L, Mu J, He X, Zhuangzhuang L, Gong T, Wang J, Min L, Lu M, Tu C. The resection of extraosseous osteosarcoma was accompanied by the occurrence of pulmonary metastasis and distal metastasis at the primary site: a case report. Front Oncol 2025; 15:1549722. [PMID: 40308507 PMCID: PMC12040817 DOI: 10.3389/fonc.2025.1549722] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/21/2024] [Accepted: 03/28/2025] [Indexed: 05/02/2025] Open
Abstract
Background Extraskeletal osteosarcoma (ESOS) is a rare and aggressive malignancy, comprising approximately 1% of all soft tissue sarcomas and 4.3% of all osteosarcomas. It predominantly affects individuals between the ages of 48 and 60, with a slightly higher incidence observed in males compared to females. The clinical diagnosis of ESOS poses a significant challenge due to its atypical presentation and overlap with other soft tissue neoplasms. Despite advances in diagnostic imaging and histopathological techniques, there is currently no consensus on the optimal treatment strategy. Case presentation We report a case of a 64-year-old Chinese woman with ESOS of the left knee for 7 years, who experienced multiple recurrences after surgical resection, accompanied by systemic multiple soft tissue metastases and lung metastases. Initially, the patient found a painless mass in the left knee, which was diagnosed as a benign soft tissue mass at a local hospital and surgically removed. However, two years after the surgery, a mass recurred around the left knee and was larger than before, prompting the patient to seek treatment at our department. The patient underwent standard surgical treatment in our department, and postoperative histopathology, genetic testing, and immunohistochemical examination all confirmed the diagnosis of ESOS. Over the course of 5 years, the patient experienced multiple recurrences, and we attempted surgical treatment combined with chemotherapy and targeted therapy. Ultimately, due to multiple tumor ruptures in the left lower limb, severe pain, and sleep disorders, the patient decided to undergo left hip disarticulation surgery. Conclusion The diagnosis and treatment of ESOS are challenging and require multimodal examination, including histological and immunohistochemical analysis. ESOS is rare, especially when it metastasizes to the distal soft tissues of the primary lesion site, which may portend a poorer clinical outcome compared to pure pulmonary metastasis. Despite current therapeutic interventions, this case still emphasizes its aggressiveness and poor prognosis.
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Affiliation(s)
- Han Liu
- Department of Orthopedics, Orthopaedic Research Institute, West China Hospital, Sichuan University, Chengdu, China
| | - Leilei Tian
- Department of Anesthesiology, West China Hospital, Sichuan University/West China School of Nursing, Sichuan University, Chengdu, China
| | - Jianhua Mu
- Department of Orthopedics, Orthopaedic Research Institute, West China Hospital, Sichuan University, Chengdu, China
| | - Xuanhong He
- Department of Orthopedics, Orthopaedic Research Institute, West China Hospital, Sichuan University, Chengdu, China
| | - Li Zhuangzhuang
- Department of Orthopedics, Orthopaedic Research Institute, West China Hospital, Sichuan University, Chengdu, China
| | - Taojun Gong
- Department of Orthopedics, Orthopaedic Research Institute, West China Hospital, Sichuan University, Chengdu, China
| | - Jingjing Wang
- Department of Endocrine, Sichuan Provincial People’s Hospital, School of Medicine, University of Electronic Science and Technology of China, Chengdu, China
| | - Li Min
- Department of Orthopedics, Orthopaedic Research Institute, West China Hospital, Sichuan University, Chengdu, China
| | - Minxun Lu
- Department of Orthopedics, Orthopaedic Research Institute, West China Hospital, Sichuan University, Chengdu, China
| | - Chongqi Tu
- Department of Orthopedics, Orthopaedic Research Institute, West China Hospital, Sichuan University, Chengdu, China
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Huo Z, Sun Y, Chang J, Li GD, Shi J, Quan C, Zhang LN, Yang TT, Shang FJ, Yang YP. Primary extraskeletal osteosarcoma of rectal mesentery: a rare case and literature review. World J Surg Oncol 2025; 23:31. [PMID: 39881285 PMCID: PMC11776156 DOI: 10.1186/s12957-025-03676-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/27/2024] [Accepted: 01/19/2025] [Indexed: 01/31/2025] Open
Abstract
BACKGROUND Extraskeletal osteosarcoma (ESOS) is a rare kind of sarcoma with a low preoperative diagnosis and a poor prognosis. ESOS arising from abdominal mesentery is extremely rare. Increasing diagnostic methods and standardizing treatment protocols are crucial issues of ESOS. CASE PRESENTATION We report the case of a 52-year-old female ESOS patient. She had a history of ovarian carcinoma (stage IIIC) surgery two years before, with five cycles of chemotherapy. A mass was found during postoperative examinations. A R0 surgical resection was performed. Post-operational pathological report plus intra-surgery findings supported a diagnosis of ESOS. She is still alive 10 months post-operationally, with routine blood and radiographical examinations. CONCLUSION Enhancing awareness of this extremely rare disease together with advancements in diagnostic methods will hopefully enable earlier recognition and initiation of treatment. Protocols for standardizing treatments require a larger multi-center collaboration and more data analysis.
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Affiliation(s)
- Zhikui Huo
- The Department of General Surgery, The Second Hospital of Jilin University, Changchun, 130041, China
| | - Yao Sun
- The Department of General Surgery, The Second Hospital of Jilin University, Changchun, 130041, China
| | - Jinghui Chang
- The Department of General Surgery, The Second Hospital of Jilin University, Changchun, 130041, China
| | - Guo-Dong Li
- The Department of General Surgery, The Second Hospital of Jilin University, Changchun, 130041, China
| | - Jian Shi
- The Department of General Surgery, The Second Hospital of Jilin University, Changchun, 130041, China
| | - Cheng Quan
- The Department of General Surgery, The Second Hospital of Jilin University, Changchun, 130041, China
| | - Li-Na Zhang
- The Department of General Surgery, The Second Hospital of Jilin University, Changchun, 130041, China
| | - Ting-Ting Yang
- The Department of General Surgery, The Second Hospital of Jilin University, Changchun, 130041, China
| | - Feng-Jia Shang
- The Department of General Surgery, The Second Hospital of Jilin University, Changchun, 130041, China
| | - Yong-Ping Yang
- The Department of General Surgery, The Second Hospital of Jilin University, Changchun, 130041, China.
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Wang DH, Zhang JL, Fan XW, Du HQ, Gao ZF, Ling DD, Cui Y, Chang MH, Zhou X. An analysis of extraskeletal osteosarcoma based on the literature. Sci Rep 2025; 15:896. [PMID: 39762371 PMCID: PMC11704034 DOI: 10.1038/s41598-025-85197-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/13/2024] [Accepted: 01/01/2025] [Indexed: 01/11/2025] Open
Abstract
Extraskeletal osteosarcoma (ESOS) is a very rare malignant tumor. This study aimed to provide more evidence about the natural history and clinical features of ESOS, and clarify the impact of chemotherapy (CT) and radiotherapy (RT) on patient survival and postoperative recurrence for the sake of gaining a better understanding about the disease. Patient/tumor characteristics, recurrence, treatment, and follow-up durations were collected by searching studies in PubMed, Web of Science, Ovid, Elsevier, SpringerLink, Chinese National Knowledge Infrastructure (CNKI), and WAN FANG Data before June 30, 2023. Data were analyzed by SPSS-26. Altogether 1287 patients diagnosed with ESOS were retrieved from the literature. Of them, follow-up data were available in 981 patients, in whom 78 patients (7.6%) had metastases and 730 patients (74.5%) had localized disease at the time of diagnosis. Of the 730 patients with localized disease, 682 (93.4%) received surgical resection. The 5-year OS in all 981 patients, 78 metastatic patients, and 682 patients with localized disease was 33.40%, 4.9% and 41.1%, respectively. Of the 682 surgical patients, 367 patients underwent surgical resection alone, 170 received surgical resection + CT, 82 patients received surgical resection + RT, and 58 patients received both adjuvants. In addition, 348 (51%) of the 682 patients developed recurrence, including local recurrence (n = 102), metastasis (n = 130), and both (n = 116). Univariate analysis of 5-year PFS and 5-year OS showed that age, tumor size, CT, metastasis, and local recurrence were significant prognostic factors in the 682 patients with localized disease. There was no significant difference in 5-year OS between osteosarcoma-type and soft tissue-type regimens (P = 0.273). Multivariate analysis of 431 patients showed that postoperative recurrence and metastasis were significant prognostic factors for survival, and CT was not a significant prognostic factor, though CT decreased the incidence of local recurrence in ≤ 45-year age group (P = 0.047). RT reduced the incidence of local recurrence in patients ≤ 45years (P = 0.035) and patients with tumors > 5 cm (P = 0.044). So, we recommend that CT should be used for patients aged ≤ 45years, and RT can be used for patients ≤ 45years or those with tumors > 5 cm for the sake of decreasing the incidence of local recurrence, which we believe would indirectly benefit the survival of ESOS patients.
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Affiliation(s)
- De-Hui Wang
- Department of Orthopedics, Jinling Hospital, Affiliated Hospital of Medical School, Nanjing University, 305 East Zhongshan Road, Nanjing, 210002, Jiangsu, China
| | - Jun-Liang Zhang
- Department of Orthopedics, Jinling Hospital, Affiliated Hospital of Medical School, Nanjing University, 305 East Zhongshan Road, Nanjing, 210002, Jiangsu, China
| | - Xiao-Wen Fan
- Jinling Clinical Medical College, Nanjing University of Chinese Medicine, Nanjing, China
| | - Hui-Qun Du
- Department of Orthopedics, Jinling Hospital, Affiliated Hospital of Medical School, Nanjing University, 305 East Zhongshan Road, Nanjing, 210002, Jiangsu, China
| | - Zi-Fan Gao
- Jinling Clinical Medical College, Nanjing University of Chinese Medicine, Nanjing, China
| | - Dong-Dong Ling
- Department of Orthopedics, Jinling Hospital, Affiliated Hospital of Medical School, Nanjing University, 305 East Zhongshan Road, Nanjing, 210002, Jiangsu, China
| | - Ying Cui
- Department of Orthopedics, Jinling Hospital, Affiliated Hospital of Medical School, Nanjing University, 305 East Zhongshan Road, Nanjing, 210002, Jiangsu, China
| | - Meng-Han Chang
- Department of Orthopedics, Jinling Hospital, Affiliated Hospital of Medical School, Nanjing University, 305 East Zhongshan Road, Nanjing, 210002, Jiangsu, China
| | - Xing Zhou
- Department of Orthopedics, Jinling Hospital, Affiliated Hospital of Medical School, Nanjing University, 305 East Zhongshan Road, Nanjing, 210002, Jiangsu, China.
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Bogojevska Doksevska M, Todorova T, Popovska D, Velkovski V, Angelovska T, Foteva M, Kunovska SK, Kocev SB, Rebok K, Samardziski M. Conoccurence of extraskeletal osteosarcoma and undiagnosed Paget disease in a 49-year-old female. J Surg Case Rep 2025; 2025:rjae826. [PMID: 39802336 PMCID: PMC11719029 DOI: 10.1093/jscr/rjae826] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/06/2024] [Accepted: 12/14/2024] [Indexed: 01/16/2025] Open
Abstract
Extraskeletal osteosarcoma (ESOS) represents a rare soft tissue entity, accounting for ⁓1% of all soft tissue malignancies. It is generally considered to have an even worse prognosis than bone osteosarcoma, therefore detailed investigations and proper treatment are required. ESOSs arising in the subcutaneous tissue are even rarer than the ones positioned in deep tissues, and they are considered to have far better outcomes. We present a case of a 49-year-old patient diagnosed with subcutaneous ESOS and Paget disease of the bone, which is not typical for the patient's age, considering that Paget disease of the bone tends to affect a population above 50 years. The coexistence of these two entities in a single patient and all their features make this case unique, and to the best of our knowledge, this is the first case reported.
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Affiliation(s)
- Milena Bogojevska Doksevska
- University Clinic for Orthopedic Diseases, 1000 Skopje, North Macedonia
- Faculty of Medicine, Ss. Cyril and Methodius University in Skopje, 1000 Skopje, Republic of North Macedonia
| | - Teodora Todorova
- University Clinic for Orthopedic Diseases, 1000 Skopje, North Macedonia
| | - Danica Popovska
- University Clinic for Orthopedic Diseases, 1000 Skopje, North Macedonia
| | - Vilijam Velkovski
- University Clinic for Orthopedic Diseases, 1000 Skopje, North Macedonia
- Faculty of Medicine, Ss. Cyril and Methodius University in Skopje, 1000 Skopje, Republic of North Macedonia
| | - Tamara Angelovska
- Faculty of Medicine, Institute of Pathology, Ss. Cyril and Methodius University, 1000 Skopje, North Macedonia
| | - Marta Foteva
- University Clinic for Orthopedic Diseases, 1000 Skopje, North Macedonia
- Faculty of Medicine, Ss. Cyril and Methodius University in Skopje, 1000 Skopje, Republic of North Macedonia
| | | | - Smiljana Bundovska Kocev
- University Institute of Radiology, Faculty of Medicine, Ss. Cyril and Methodius University, 1000 Skopje, North Macedonia
| | - Katerina Rebok
- Laboratory of Cytology, Histology and Embryology, Faculty of Natural Sciences and Mathematics, Institute of Biology, Ss. Cyril and Methodius University, 1000 Skopje, North Macedonia
| | - Milan Samardziski
- University Clinic for Orthopedic Diseases, 1000 Skopje, North Macedonia
- Faculty of Medicine, Ss. Cyril and Methodius University in Skopje, 1000 Skopje, Republic of North Macedonia
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Campos F, Téres R, Sebio A, Bettim BB, Martinez-Trufero J. Survival Differences of Patients with Resected Extraskeletal Osteosarcoma Receiving Two Different (Neo)Adjuvant Chemotherapy Regimens: A Systematic Review and Meta-analysis. Clin Oncol (R Coll Radiol) 2023; 35:e720-e727. [PMID: 37777356 DOI: 10.1016/j.clon.2023.09.009] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/01/2023] [Accepted: 09/14/2023] [Indexed: 10/02/2023]
Abstract
AIMS Extraskeletal osteosarcoma (ESOS) is a malignant tumour developing in soft tissues, characterised by the production of osteoid or bone matrix by tumour cells. The standard treatment for localised ESOS is wide resection. Radiotherapy and chemotherapy are usually incorporated into the management of patients. Two types of chemotherapy regimen are mostly used: an osteosarcoma-type chemotherapy, based on cisplatin, and a soft-tissue sarcoma (STS)-type chemotherapy, using the combination of doxorubicin and ifosfamide. To investigate the difference in survival between these two chemotherapy regimens, a systematic review of studies reporting the 5-year disease-free survival (DFS) rates among patients with ESOS submitted to surgery and who received (neo)adjuvant chemotherapy with osteosarcoma-type or STS-type chemotherapy was carried out. MATERIALS AND METHODS Of the 401 articles identified by systematically searching the PubMed, Embase and Cochrane Central Register of Controlled Trials databases, six retrospective studies were included in the final analysis. In total, 319 patients with localised/resected ESOS were included in the study. RESULTS Our meta-analysis showed a benefit in 5-year DFS favouring the use of osteosarcoma-type chemotherapy (relative risk = 1.32, 95% confidence interval 1.03-1.69; P = 0.54); I2 heterogeneity was 0%. The 5-year DFS rate was 56.3% (95% confidence interval 48.3-64.3) with osteosarcoma-type chemotherapy and 45.2% (95% confidence interval 34.5-55.9) with STS-type chemotherapy, with I2 heterogeneity of 27% and 0%, respectively. CONCLUSIONS Our analysis suggests that there may be a difference regarding the type of (neo)adjuvant chemotherapy regimen used in the treatment of patients with resected ESOS in favour of osteosarcoma-type chemotherapy. Future studies evaluating the role of this treatment modality in this scenario need to consider the type of chemotherapy regimen when comparing with an arm of surgery with/without radiotherapy alone.
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Affiliation(s)
- F Campos
- Medical Oncology Department, Soft Tissue Sarcoma and Bone Tumors Reference Center, A.C. Camargo Cancer Center, São Paulo, Brazil; Hospital Municipal da Vila Santa Catarina/Hospital Israelita Albert Einstein, São Paulo, Brazil.
| | - R Téres
- Medical Oncology Department, Hospital Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain
| | - A Sebio
- Medical Oncology Department, Hospital Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain
| | - B B Bettim
- International Center of Research CIPE, A.C. Camargo Cancer Center, Sao Paulo, Brazil
| | - J Martinez-Trufero
- Medical Oncology Department, Hospital Universitario Miguel Servet, Zaragoza, Spain
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8
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Dabas SK, Menon NN, Ranjan R, Sharma A, Gurung B, Shukla H, Dawar H, Tiwari S, Sinha A, Singal R, Bhatti SS, Dua A. A Rare Case of Extraosseous Osteosarcoma (EOS) of Parotid Gland. Indian J Otolaryngol Head Neck Surg 2023; 75:1215-1220. [PMID: 37275042 PMCID: PMC10234941 DOI: 10.1007/s12070-023-03497-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/03/2022] [Accepted: 01/16/2023] [Indexed: 01/31/2023] Open
Abstract
Extraosseous osteosarcoma is a rare malignant soft tissue neoplasm, and extraosseous osteosarcoma of the parotid gland is very rare. It has a very aggressive course, and there are no standardized treatment guidelines. We report the case of a 20 year old male patient who presented with history of right neck swelling since 6 years for which he had undergone right parotid surgery 5 years ago. The final histopathological report indicated that the mass was a pleomorphic adenoma. One year after the first surgery, the patient experienced recurrence of swelling over the operated site, and the size of the swelling has been increasing gradually since then. He was evaluated clinically, and a large mass was noted over the upper aspect of the right upper neck, extending to the occipital and parotid regions. An MRI scan was done which showed a 12 × 10 × 8 cm lesion centred in the right parotid gland, involving paraspinal muscles, C1-C2 vertebrae and extending into the parapharyngeal space. FNAC of the lesion showed features of pleomorphic adenoma. The patient underwent a complete excision of the tumour. The patient's post-operative period was uneventful. The final histopathological report of the patient was extraosseous osteosarcoma of the parotid gland. The patient was referred for adjuvant radiotherapy. He has been on regular follow-up for the past 6 months and has shown no sign of recurrence. EOS is an extremely rare tumour of the head and neck region which often requires extensive surgical resection with or without adjuvant radiotherapy. It has a high rate of local recurrence and a very low disease free survival. Such patients should be kept on a close follow-up.
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Affiliation(s)
- Surendra K Dabas
- Department of Surgical oncology, BLK- MAX Super speciality hospital, Pusa road, Rajendra Place, Delhi, 110005 India
| | - Nandini N Menon
- Department of Surgical oncology, BLK- MAX Super speciality hospital, Pusa road, Rajendra Place, Delhi, 110005 India
| | - Reetesh Ranjan
- Department of Surgical oncology, BLK- MAX Super speciality hospital, Pusa road, Rajendra Place, Delhi, 110005 India
| | - Ashwani Sharma
- Department of Surgical oncology, BLK- MAX Super speciality hospital, Pusa road, Rajendra Place, Delhi, 110005 India
| | - Bikas Gurung
- Department of Surgical oncology, BLK- MAX Super speciality hospital, Pusa road, Rajendra Place, Delhi, 110005 India
| | - Himanshu Shukla
- Department of Surgical oncology, BLK- MAX Super speciality hospital, Pusa road, Rajendra Place, Delhi, 110005 India
| | - Hitesh Dawar
- Department of Surgical oncology, BLK- MAX Super speciality hospital, Pusa road, Rajendra Place, Delhi, 110005 India
| | - Sukirti Tiwari
- Department of Surgical oncology, BLK- MAX Super speciality hospital, Pusa road, Rajendra Place, Delhi, 110005 India
| | - Ajit Sinha
- Department of Surgical oncology, BLK- MAX Super speciality hospital, Pusa road, Rajendra Place, Delhi, 110005 India
| | - Rishu Singal
- Department of Radiodiagnosis, BLK- MAX Super speciality hospital, Pusa road, Rajendra Place, Delhi, 110005 India
| | - Sahibinder Singh Bhatti
- Department of Histopathology, BLK- MAX Super speciality hospital, Pusa road, Rajendra Place, Delhi, 110005 India
| | - Aditi Dua
- Department of Surgical oncology, BLK- MAX Super speciality hospital, Pusa road, Rajendra Place, Delhi, 110005 India
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9
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Abou Shaar B, El-Karim GA, Alsaied AR, Almalki N, Ashraf N, Almalki A, Duggal R, Munir S. Beyond the bones: Extraskeletal osteosarcoma of the thigh. Radiol Case Rep 2023; 18:2126-2135. [PMID: 37089974 PMCID: PMC10113790 DOI: 10.1016/j.radcr.2023.03.009] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/11/2023] [Revised: 02/28/2023] [Accepted: 03/02/2023] [Indexed: 04/08/2023] Open
Abstract
Extraskeletal osteosarcoma (ESOS) is a rare malignant mesenchymal soft tissue tumor that usually arises in the lower extremities. It is typically a high-grade malignancy that represents only around 1%-2% of all soft tissue sarcomas and 2%-4% of all osteosarcomas. In this report, we describe a case of a 67-year-old female who presented with a 4-day history of a painless lump in her posterior right thigh. Workup utilizing different imaging modalities yielded a diagnosis of ESOS. The radiologic features of ESOS, as well as the current treatment paradigm and prognosis of this rare malignancy, will be discussed based on a review of the literature.
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Affiliation(s)
- Bader Abou Shaar
- College of Medicine, Alfaisal University, Riyadh, Saudi Arabia
- Department of Diagnostic Imaging, Bluewater Health, Sarnia, ON, Canada
- Department of Medical Imaging, Juravinski Hospital, Hamilton, ON, Canada
| | | | | | - Nadeem Almalki
- Faculty of Math, University of Waterloo, Waterloo, ON, Canada
| | - Nader Ashraf
- College of Medicine, Alfaisal University, Riyadh, Saudi Arabia
| | - Ameera Almalki
- Department of Diagnostic Imaging, Bluewater Health, Sarnia, ON, Canada
| | - Rishi Duggal
- Department of Diagnostic Imaging, Bluewater Health, Sarnia, ON, Canada
- Department of Medical Imaging, Western University, London, ON, Canada
| | - Sohaib Munir
- Department of Medical Imaging, Juravinski Hospital, Hamilton, ON, Canada
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10
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Habeeb O, Weigelt MA, Goldblum JR, Ko JS, Habermehl G, Rubin BP, Billings SD. Primary cutaneous extraskeletal osteosarcoma: a series of 16 cases. Pathology 2023; 55:315-323. [PMID: 36567163 DOI: 10.1016/j.pathol.2022.10.002] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/31/2022] [Revised: 10/03/2022] [Accepted: 10/11/2022] [Indexed: 12/03/2022]
Abstract
Extraskeletal osteosarcoma (EOS) is a high grade soft tissue tumour characterised by the production of malignant osteoid, without attachment/involvement of underlying bone/periosteum. Rarely, EOS presents as a cutaneous tumour. The clinical behaviour of primary cutaneous EOS (PC-EOS) remains incompletely characterised. Herein we present the largest case series of PC-EOS reported to date. Sixteen PC-EOS cases from the archives/consultation files were retrieved (male:female 1:1; age 31-96 years, mean age 66 years). The tumours measured 1-10 cm (mean 3.2 cm) and were located on the lower extremity (7), head (6), upper extremity (2), and trunk (1). They consisted of pleomorphic, spindled-to-epithelioid cells, with fascicular, nodular, or sheet-like growth patterns and foci of malignant osteoid. Immunohistochemistry did not reveal specific lines of differentiation, and there was no evidence of other tumour types. A literature review was conducted to identify all well characterised cases of PC-EOS. A combined analysis of present and past cases was performed to determine overall trends in clinical characteristics and outcomes. The mean follow-up period was 23.9 months, during which 67.5% of patients experienced progression-free survival and 18% of patients died of disease. Rates of local recurrence and metastasis were 10% and 25%, respectively, approximately double past estimates. These data suggest that the prognosis of PC-EOS is less favourable than previously thought. The differential diagnosis includes benign entities (e.g., ossifying pyogenic granuloma) and malignant neoplasms with heterologous osteosarcomatous differentiation (e.g., carcinosarcoma, transdifferentiated melanoma). Wide excision remains the standard of care, and the role of chemotherapy and radiation remains inconclusive. Recognition of this rare entity can facilitate prompt diagnosis and appropriate treatment.
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Affiliation(s)
- Omar Habeeb
- Department of Histopathology, Middlemore Hospital, Auckland, New Zealand
| | | | - John R Goldblum
- Department of Pathology, Cleveland Clinic Foundation, Cleveland, OH, USA
| | - Jennifer S Ko
- Department of Pathology, Cleveland Clinic Foundation, Cleveland, OH, USA
| | | | - Brian P Rubin
- Department of Pathology, Cleveland Clinic Foundation, Cleveland, OH, USA
| | - Steven D Billings
- Department of Pathology, Cleveland Clinic Foundation, Cleveland, OH, USA.
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11
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Hesni S, Lindsay D, O'Donnell P, Saifuddin A. Extra-skeletal osteosarcoma: a review. Skeletal Radiol 2023; 52:633-648. [PMID: 36194245 DOI: 10.1007/s00256-022-04193-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/29/2022] [Revised: 09/26/2022] [Accepted: 09/26/2022] [Indexed: 02/02/2023]
Abstract
Extra-skeletal osteosarcoma is a rare malignant soft tissue sarcoma which can cause a diagnostic challenge due to its non-specific presentation and soft tissue mineralisation, thus potentially mimicking conditions such as myositis ossificans. This review will outline the demographics, clinical presentation, key imaging features, differential diagnosis, management and outcomes of extra-skeletal osteosarcoma and serve as a reference to radiologists and other clinicians involved in the care of patients with soft tissue tumours and tumour-like lesions.
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Affiliation(s)
- Susan Hesni
- Department of Radiology, Royal National Orthopaedic Hospital (RNOH), Stanmore, UK.
| | - Daniel Lindsay
- Department of Histopathology, Royal National Orthopaedic Hospital (RNOH), Stanmore, UK
| | - Paul O'Donnell
- Department of Radiology, Royal National Orthopaedic Hospital (RNOH), Stanmore, UK
| | - Asif Saifuddin
- Department of Radiology, Royal National Orthopaedic Hospital (RNOH), Stanmore, UK
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12
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Crombé A, Spinnato P, Righi A, Leopardi MP, Carpenzano M, Izzo F, Parmeggiani A, Linck PA, Perret R, Cesari M, Longhi A, Miceli M, Kind M, Bianchi G. Imaging presentation of extraskeletal osteosarcomas on CT and MRI and correlation with patients outcome: A two-center retrospective study of 54 patients. Diagn Interv Imaging 2023; 104:297-306. [PMID: 36813659 DOI: 10.1016/j.diii.2023.01.009] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/22/2022] [Revised: 01/18/2023] [Accepted: 01/26/2023] [Indexed: 02/24/2023]
Abstract
PURPOSE The purpose of this study was to analyze the imaging features of extraskeletal osteosarcomas (ESOS) on computed tomography (CT) and magnetic resonance imaging (MRI) and to investigate their associations with overall survival (OS) using uni- and multivariable survival analyses. MATERIALS AND METHODS This two-center retrospective study included all consecutive adult patients between 2008 and 2021 with histopathologically-proven ESOS who underwent pre-treatment CT and/or MRI. Clinical and histological characteristics, ESOS presentation on CT and MRI, treatment and outcomes were reported. Survival analyses were performed using Kaplan-Meier analysis and Cox regressions. Associations between imaging features and OS were searched using uni- and multivariable analyses. RESULTS Fifty-four patients were included (30/54 [56%] men, median age: 67.5 years). Twenty-four died of ESOS (median OS: 18 months). ESOS were mostly deep-seated (46/54, 85%) in the lower limb (27/54, 50%) with a median size of 95 mm (interquartile range: 64, 142; range: 21-289 mm). Mineralization was seen on 26/42 (62%) patients, mainly gross-amorphous (18/26; 69%). ESOS were generally highly heterogeneous on T2-weighted images (38/48; 79%) and contrast-enhanced (CE) T1-weighted images (29/40; 72%), with necrosis (39/40; 97%), well-defined or focally infiltrative margins (39/47; 83%), with moderate peritumoral edema (39/47; 83%) and rim-like peripheral enhancement (17/40; 42%). Size, location, mineralization on CT, signal intensity heterogeneity on T1-, T2- and CE-T1-weighted images and hemorragic signal on MRI were associated with poorer OS (range of log-rank P = 0.0069-0.0485). At multivariable analysis, hemorragic signal and signal intensity heterogeneity on T2-weighted images remained predictive for poorer OS (hazard ratio [HR] = 2.68, P = 0.0299; HR = 9.85, P = 0.0262, respectively) CONCLUSION: ESOS typically presents as mineralized heterogeneous and necrotic soft tissue tumor with a possible rim-like enhancement and limited peritumoral abnormalities. MRI may help estimate outcome of patients with ESOS.
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Affiliation(s)
- Amandine Crombé
- Department of Musculoskeletal Imaging, Pellegrin University Hospital, 33000, Bordeaux, France; Department of Oncologic Imaging, Institut Bergonié, 33076, Bordeaux, France; Models in Oncology (MONC) Team, INRIA Bordeaux Sud-Ouest, CNRS UMR 5251 & Bordeaux University, 33400, Talence, France
| | - Paolo Spinnato
- Diagnostic and Interventional Radiology, IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy
| | - Alberto Righi
- Department of Pathology, IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy
| | | | - Maria Carpenzano
- Diagnostic and Interventional Radiology, IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy
| | - Federica Izzo
- Diagnostic and Interventional Radiology, IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy
| | - Anna Parmeggiani
- Diagnostic and Interventional Radiology, IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy
| | | | - Raul Perret
- Department of Biopathology, Comprehensive Cancer Center, 33000, Bordeaux, France
| | - Marilena Cesari
- Osteoncology, Bone and Soft Tissue Sarcomas, and Innovative Therapies, IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy
| | - Alessandra Longhi
- Osteoncology, Bone and Soft Tissue Sarcomas, and Innovative Therapies, IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy
| | - Marco Miceli
- Diagnostic and Interventional Radiology, IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy
| | - Michèle Kind
- Department of Oncologic Imaging, Institut Bergonié, 33076, Bordeaux, France
| | - Giuseppe Bianchi
- Department of Orthopedic Oncology, IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy
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13
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Tsukamoto S, Mavrogenis AF, Angelelli L, Righi A, Filardo G, Kido A, Honoki K, Tanaka Y, Tanaka Y, Errani C. The Effect of Adjuvant Chemotherapy on Localized Extraskeletal Osteosarcoma: A Systematic Review. Cancers (Basel) 2022; 14:cancers14102559. [PMID: 35626164 PMCID: PMC9139294 DOI: 10.3390/cancers14102559] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/23/2022] [Revised: 05/20/2022] [Accepted: 05/20/2022] [Indexed: 12/10/2022] Open
Abstract
(1) Background: Extraskeletal osteosarcoma (ESOS) is a malignant tumor characterized by the production of bone or bone matrix by tumor cells without any continuity into the skeletal bones. The standard treatment for localized ESOS is wide resection; however, the effect of (neo)adjuvant chemotherapy remains unclear. To investigate the effect of (neo)adjuvant chemotherapy for localized ESOS, we conducted a systematic review of studies comparing the 5-year disease-free survival rate between patients who underwent surgery combined with (neo)adjuvant chemotherapy and those who underwent surgery alone. (2) Methods: Of the 210 studies identified by systematically searching the PubMed, Embase, and Cochrane Central Register of Controlled Trials databases, 12 were included in the final analysis. These 12 articles were not randomized controlled trials, but retrospective studies. In total, 761 patients with localized ESOS were included in this study. (3) Results: The 5-year disease-free survival rate was 47.9% (187 of 390 patients) in the surgery and (neo)adjuvant chemotherapy group and 40.4% (150 of 371 patients) in the surgery alone group. The overall pooled odds ratio was 1.23 (95% confidence interval, 0.69-2.19; p = 0.479) and the heterogeneity I2 was 37%. (4) Conclusions: The effect of adjuvant chemotherapy on localized ESOS seems to be limited. Therefore, routine use of adjuvant chemotherapy for localized ESOS should be avoided. However, further randomized controlled trials are required to confirm these results.
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Affiliation(s)
- Shinji Tsukamoto
- Department of Orthopaedic Surgery, Nara Medical University, 840, Shijo-Cho, Kashihara 634-8521, Japan; (S.T.); (K.H.); (Y.T.)
| | - Andreas F. Mavrogenis
- First Department of Orthopaedics, School of Medicine, National and Kapodistrian University of Athens, Athens 15562, Greece
- Correspondence: ; Tel./Fax: +30-210-654-2800
| | - Lucia Angelelli
- Applied and Translational Research Center, IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy; (L.A.); (G.F.)
| | - Alberto Righi
- Department of Pathology, IRCCS Istituto Ortopedico Rizzoli, Via di Barbiano 1/10, 40136 Bologna, Italy;
| | - Giuseppe Filardo
- Applied and Translational Research Center, IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy; (L.A.); (G.F.)
| | - Akira Kido
- Department of Rehabilitation Medicine, Nara Medical University, 840, Shijo-Cho, Kashihara 634-8521, Japan;
| | - Kanya Honoki
- Department of Orthopaedic Surgery, Nara Medical University, 840, Shijo-Cho, Kashihara 634-8521, Japan; (S.T.); (K.H.); (Y.T.)
| | - Yuu Tanaka
- Department of Rehabilitation Medicine, Wakayama Professional University of Rehabilitation, 3-1, Minamoto-Cho, Wakayama 640-8222, Japan;
| | - Yasuhito Tanaka
- Department of Orthopaedic Surgery, Nara Medical University, 840, Shijo-Cho, Kashihara 634-8521, Japan; (S.T.); (K.H.); (Y.T.)
| | - Costantino Errani
- Department of Orthopaedic Oncology, IRCCS Istituto Ortopedico Rizzoli, Via Pupilli 1, 40136 Bologna, Italy;
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14
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Nie X, Fu W, Li C, Lu L, Li W. Primary extraskeletal osteosarcoma of sigmoid mesocolon: a case report and a review of the literature. World J Surg Oncol 2021; 19:267. [PMID: 34479594 PMCID: PMC8417981 DOI: 10.1186/s12957-021-02337-9] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/23/2021] [Accepted: 07/17/2021] [Indexed: 11/10/2022] Open
Abstract
Background Extraskeletal osteosarcoma (ESOS) is a rare mesenchymal malignancy, which produces osteoid, bone, or chondroid material and is located in the soft tissue without attachment to skeletal bones and periosteum. One of the things that ESOS originated from mesentery is much rarer. Case presentation A 75-year female had a history of pain in the left lower abdomen for more than 4 months. Abdominal computerized tomography (CT) and magnetic resonance imaging revealed a large, irregular, and solid-cystic mass (largest diameter was 11.5 cm). The tumor was radically removed during an open operation. It was composed of abundant osteoid and polyhedral-shaped tumor cells with high atypia and high mitotic activity microscopically. The final pathological diagnosis was osteoblastic osteosarcoma, arising from the sigmoid mesocolon with negative margins. A 9-month follow-up by CT exhibited signs of peritoneal metastasis. Conclusions Given the rarity of cases of mesenteric ESOS, diagnosis mainly depended on pathology findings or should be taken into consideration when the mesenteric mass was found. Its most effective treatment had not been determined, with surgical excision being generally accepted. Ensuring negative surgical margins may be an important factor affecting prognosis.
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Affiliation(s)
- Xinyang Nie
- The Graduate School, Tianjin Medical University, Tianjin, China.,Department of General Surgery, Tianjin Medical University General Hospital, 154, Anshan Road, Heping District, Tianjin, 300052, China
| | - Weihua Fu
- Department of General Surgery, Tianjin Medical University General Hospital, 154, Anshan Road, Heping District, Tianjin, 300052, China.
| | - Chuan Li
- Department of General Surgery, Tianjin Medical University General Hospital, 154, Anshan Road, Heping District, Tianjin, 300052, China
| | - Li Lu
- Department of General Surgery, Tianjin Medical University General Hospital, 154, Anshan Road, Heping District, Tianjin, 300052, China
| | - Weidong Li
- Department of General Surgery, Tianjin Medical University General Hospital, 154, Anshan Road, Heping District, Tianjin, 300052, China.
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15
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Kattepur AK, Gulia A, Jones RL, Rastogi S. Extraskeletal osteosarcomas: current update. Future Oncol 2021; 17:825-835. [PMID: 33533642 DOI: 10.2217/fon-2020-0802] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/21/2022] Open
Abstract
Extraskeletal osteosarcoma is a very infrequently diagnosed soft-tissue sarcoma subtype which has identical histological features to bone osteosarcoma. However, its demographics, presentation, radiology and treatment strategy differ from those of osteosarcoma. Its diagnosis can be at times challenging due to radiological and pathological mimics which have more common incidence. A multimodality approach is essential for optimizing the outcomes in extraskeletal osteosarcoma. Although there are certain caveats on inclusion of adjuvant therapies (radiotherapy and chemotherapy), in all cases surgical resection with wide local margins is considered the gold standard for adequate local control. The outcome in advanced disease remains dismal and there is a huge unmet need for prospective studies addressing the optimal treatment strategy. In this article, we review the evidence available for the management of extraskeletal osteosarcoma.
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Affiliation(s)
- Abhay K Kattepur
- Department of Surgical Oncology, Sri Devaraj Urs Medical College, Tamaka, Kolar 563101, Karnataka, India
| | - Ashish Gulia
- Department of Surgical Oncology, Bone & Soft Tissue Services, Tata Memorial Hospital & Homi Bhabha National Institute (HBNI), Dr Ernst Borges Marg, Parel, Mumbai, 400012, India
| | - Robin L Jones
- Royal Marsden Hospital, NHS Trust, Fulham Road, London, SW3 6JJ, UK
| | - Sameer Rastogi
- Department of Medical Oncology, All India Institute of Medical Sciences, Sri Aurobindo Marg, Ansari Nagar, Ansari Nagar East, New Delhi, 110029, India
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16
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Qi L, Wan L, Ren X, Zhang W, Tu C, Li Z. The Role of Chemotherapy in Extraskeletal Osteosarcoma: A Propensity Score Analysis of the Surveillance Epidemiology and End Results (SEER) Database. Med Sci Monit 2020; 26:e925107. [PMID: 32792473 PMCID: PMC7446278 DOI: 10.12659/msm.925107] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/14/2020] [Accepted: 05/15/2020] [Indexed: 12/03/2022] Open
Abstract
BACKGROUND Incidence of extraskeletal osteosarcoma (ESOS) is extremely low and the prognosis remains unclear. We conducted this study to explore prognostic factors and the role of chemotherapy in ESOS. MATERIAL AND METHODS We screened data from the Surveillance Epidemiology and End Results (SEER) database (1975-2016). Three hundred ten patients with ESOS were included and 49.4% (107/310) of them underwent chemotherapy. We performed logistic regression analysis to investigate potential factors determining selection of chemotherapy. An inverse probability of treatment weighting (IPTW) and propensity score matching (PSM)-adjusted Kaplan-Meier curve was created and log-rank test and Cox regression analysis were performed to compare overall survival (OS) and cancer-specific survival (CSS) in patients treated with and without chemotherapy. Subgroup analysis also was conducted based on age, tumor site, stage, size, and surgery. RESULTS Chemotherapy in ESOS was not associated with improved OS in the unmatched cohort (HR, 0.764; 95% CI, 0.555-1.051; p=0.098). The insignificant treatment effect of chemotherapy was also noted in IPTW-adjusted (HR, 0.737; 95% CI, 0.533-1.021; p=0.066) and PSM-adjusted (HR, 0.804; 95% CI, 0.552-1.172; p=0.257) Cox regression analysis. The insignificant treatment effect was consistent across all subgroups and there was no significant heterogeneity of chemotherapy effect (all p for interaction >0.05). CONCLUSIONS The study suggested that chemotherapy has no significant benefit on prognosis of patients with ESOS. These findings should be considered when making treatment decisions about patients with ESOS.
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Affiliation(s)
- Lin Qi
- Department of Orthopedics, The Second Xiangya Hospital, Central South University, Changsha, Hunan, P.R. China
- Hunan Key Laboratory of Tumor Models and Individualized Medicine, The Second Xiangya Hospital, Changsha, Hunan, P.R. China
| | - Lu Wan
- Department of Orthopedics, The Second Xiangya Hospital, Central South University, Changsha, Hunan, P.R. China
- Hunan Key Laboratory of Tumor Models and Individualized Medicine, The Second Xiangya Hospital, Changsha, Hunan, P.R. China
- Vaccine and Immunotherapy Center, Massachusetts General Hospital and Harvard Medical School, Charlestown, MA, U.S.A
| | - Xiaolei Ren
- Department of Orthopedics, The Second Xiangya Hospital, Central South University, Changsha, Hunan, P.R. China
- Hunan Key Laboratory of Tumor Models and Individualized Medicine, The Second Xiangya Hospital, Changsha, Hunan, P.R. China
| | - Wenchao Zhang
- Department of Orthopedics, The Second Xiangya Hospital, Central South University, Changsha, Hunan, P.R. China
- Hunan Key Laboratory of Tumor Models and Individualized Medicine, The Second Xiangya Hospital, Changsha, Hunan, P.R. China
| | - Chao Tu
- Department of Orthopedics, The Second Xiangya Hospital, Central South University, Changsha, Hunan, P.R. China
- Hunan Key Laboratory of Tumor Models and Individualized Medicine, The Second Xiangya Hospital, Changsha, Hunan, P.R. China
| | - Zhihong Li
- Department of Orthopedics, The Second Xiangya Hospital, Central South University, Changsha, Hunan, P.R. China
- Hunan Key Laboratory of Tumor Models and Individualized Medicine, The Second Xiangya Hospital, Changsha, Hunan, P.R. China
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17
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Wang T, Liao S, Ding X, Anil KC, Huang Q, Lin C, Mo J, Tang H, Liu Y. Intraperitoneal extraosseous osteosarcoma: a case report and literatures review. BMC Musculoskelet Disord 2020; 21:452. [PMID: 32653041 PMCID: PMC7353749 DOI: 10.1186/s12891-020-03429-5] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/19/2019] [Accepted: 06/16/2020] [Indexed: 11/18/2022] Open
Abstract
Background To investigate the clinical imaging manifestations, diagnosis and treatment of intraperitoneal extraosseous osteosarcoma. Case presentation A 52-year-old male patient with intraperitoneal extraosseous osteosarcoma was retrospectively analyzed. He suffered from left lower abdominal pain accompanied by mass for 6 months. On abdominal CT scan, multiple patchy and banded calcification were found. The largest is about six centimeters in diameter and underwent mass resection. Postoperative pathology revealed retroperitoneal osteosarcoma. The reported intraperitoneal extraosseous osteosarcoma was analyzed and the related literature was reviewed. Two years after operation, the patients had recurrence of the tumors and invaded sigmoid colon, peritoneum and bladder. Palliative operation was performed to remove the tumors in the bladder and transverse colostomy was performed. The patients were followed up by telephone and died 2 months after the second operation. Conclusions Intraperitoneal extraosseous osteosarcoma has a low incidence and has no specific imaging features. Surgical resection is the main treatment and the prognosis is poor.
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Affiliation(s)
- Tiantian Wang
- Departments of Orthopedics, The First Affiliated Hospital of Guangxi Medical University, Nanning, 530021, Guangxi, China
| | - Shijie Liao
- Departments of Orthopedics, The First Affiliated Hospital of Guangxi Medical University, Nanning, 530021, Guangxi, China.,Research Centre for Regenerative Medicine, Guangxi Key Laboratory of Regenerative Medicine, Guangxi Medical University, 22 Shuangyong Road, Nanning, Guangxi, China
| | - Xiaofei Ding
- Departments of Orthopedics, The First Affiliated Hospital of Guangxi Medical University, Nanning, 530021, Guangxi, China.,Research Centre for Regenerative Medicine, Guangxi Key Laboratory of Regenerative Medicine, Guangxi Medical University, 22 Shuangyong Road, Nanning, Guangxi, China
| | - K C Anil
- Departments of Orthopedics, The First Affiliated Hospital of Guangxi Medical University, Nanning, 530021, Guangxi, China
| | - Qian Huang
- Research Centre for Regenerative Medicine, Guangxi Key Laboratory of Regenerative Medicine, Guangxi Medical University, 22 Shuangyong Road, Nanning, Guangxi, China
| | - Chengsen Lin
- Departments of Orthopedics, The First Affiliated Hospital of Guangxi Medical University, Nanning, 530021, Guangxi, China
| | - Jianming Mo
- Departments of Orthopedics, The First Affiliated Hospital of Guangxi Medical University, Nanning, 530021, Guangxi, China
| | - Haijun Tang
- Departments of Orthopedics, The First Affiliated Hospital of Guangxi Medical University, Nanning, 530021, Guangxi, China
| | - Yun Liu
- Departments of Orthopedics, The First Affiliated Hospital of Guangxi Medical University, Nanning, 530021, Guangxi, China.
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18
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Saadaat R, Abdul-Ghafar J, Ud Din N, Haidary AM. Anal extraskeletal osteosarcoma in a man: a case report and review of the literature. J Med Case Rep 2020; 14:51. [PMID: 32312303 PMCID: PMC7171741 DOI: 10.1186/s13256-020-02365-1] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/05/2019] [Accepted: 02/26/2020] [Indexed: 11/10/2022] Open
Abstract
BACKGROUND Osteosarcoma is a common malignancy of bone that usually occurs in individuals in the age range of 0-24 years. Extraskeletal osteosarcoma is a rare tumor presentation which originates in non-bony tissues. Extraskeletal osteosarcoma comprises 2-5% of all osteosarcomas and less than 1% of all soft tissue sarcomas. As compared to bone-derived osteosarcoma, extraskeletal osteosarcoma occurs in older age groups. Extraskeletal osteosarcoma has a poorer prognosis than bone osteosarcoma. To the best of our knowledge, this is the first case of extraskeletal osteosarcoma in the anal region. CASE PRESENTATION A 70-year-old Hazara man presented to a private hospital with the chief complaints of constipation, bloody defecation, and pain during defecation of 1.5 months' duration. His past history was unremarkable. A digital rectal examination showed a solid growth in the middle part of his anus. A colonoscopic examination was done and showed a solid mass in his anal region. A computed tomography scan revealed an irregular mural thickening in the anal canal with heterogeneous enhancement. The maximum length of the involved segment was measured to be 4.5 cm. No suspicious lesions were noted in other organs. An abdominoperineal resection was performed on our patient. A 22 cm in length resected segment of his colon, consisting of the lower sigmoid, rectum, and anus was sent to us for histopathological examination. Gross examination revealed a polypoid dark-gray tumor measuring 5 × 3 × 2 cm. The cut section revealed gray and white appearance with firm-to-hard consistency and foci of ossification. Microscopic examination revealed normal anorectal mucosa and a spindle cell malignant neoplasm with osteoid formations. No evidence of epithelial carcinoma was noted. Immunohistochemical stains were positive for stabilin-2 and negative for cytokeratin, which confirmed the diagnosis of osteosarcoma. CONCLUSION Extraskeletal osteosarcoma of the colon is rare and presence of the tumor in the rectum and anal region is extremely rare. Radiology, colonoscopy, and histopathology with immunostaining are required for the diagnosis. The accurate diagnosis of extraskeletal osteosarcoma is important as it has a different regimen of treatment with poorer prognosis compared to primary osteosarcoma of the bone.
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Affiliation(s)
- Ramin Saadaat
- Department of Pathology and Laboratory Medicine, French Medical Institute for Mothers and Children (FMIC), Kabul, Afghanistan
| | - Jamshid Abdul-Ghafar
- Department of Pathology and Laboratory Medicine, French Medical Institute for Mothers and Children (FMIC), Kabul, Afghanistan.
| | - Nasir Ud Din
- Department of Pathology and Laboratory Medicine, Aga Khan University Hospital (AKU-H), Karachi, Pakistan
| | - Ahmed Maseh Haidary
- Department of Pathology and Laboratory Medicine, French Medical Institute for Mothers and Children (FMIC), Kabul, Afghanistan
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19
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Araki Y, Yamamoto N, Hayashi K, Takeuchi A, Miwa S, Igarashi K, Taniguchi Y, Yonezawa H, Morinaga S, Nojima T, Tsuchiya H. Cystic extraskeletal osteosarcoma: Three case reports and review of the literature. Mol Clin Oncol 2020; 12:468-474. [PMID: 32257205 DOI: 10.3892/mco.2020.2015] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/14/2019] [Accepted: 01/31/2020] [Indexed: 11/06/2022] Open
Abstract
Extraskeletal osteosarcoma is a rare soft tissue sarcoma and typically appears as a solid mass with variable mineralization. A few cases of extraskeletal osteosarcoma have shown extensive hemorrhagic changes or bleeding due to its high-grade malignancy; however, to the best of our knowledge, no previous reports have described the pathological characteristics of tumors with non-hemorrhagic cystic change. The present report discusses three cases involving cystic lesions with a solid area at the periphery that arose in soft tissues. The large cystic spaces contained only yellowish-brown fluid and little or no blood, with no clear pathological necrotic tissue. The solid component comprised a focal area of osteoid production by highly anaplastic sarcomatous cells. All of the cases showed high-grade malignancy histologically, and the average maximum diameter was approximately 175 mm. The tumors were located at the adductor muscles in two cases and at the intermuscular region of the hamstring muscles in one case. All of the patients consulted a doctor after more than half a year had passed since the recognition of swelling or a mass on their affected extremities. Surgical treatment was performed for all patients. One patient had lymph node metastasis, and another had lymphoedema after surgery, but no histological invasion to the lymphatic ducts was observed in the excised specimen. Degenerative changes in the fluid content over a long time-course as a result of tumor bleeding or necrosis, rather than lymphorrhea by lymphatic channel invasion of the tumor, might have contributed to the formation of the cystic lesion. Only 1 patient who underwent chemotherapy remained clinically disease-free over 10 years after surgery. Chemotherapy regimens for osteosarcoma rather than those for soft tissue sarcoma are mostly effective for extraskeletal osteosarcoma, so the diagnosis by a biopsy is essential. It is important to consider extraskeletal osteosarcoma in the differential diagnosis of soft tissue tumor with cystic form and calcification and a long clinical course before consulting a doctor.
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Affiliation(s)
- Yoshihiro Araki
- Department of Orthopedic Surgery, Graduate School of Medical Sciences, Kanazawa, Ishikawa 920-8641, Japan
| | - Norio Yamamoto
- Department of Orthopedic Surgery, Graduate School of Medical Sciences, Kanazawa, Ishikawa 920-8641, Japan
| | - Katsuhiro Hayashi
- Department of Orthopedic Surgery, Graduate School of Medical Sciences, Kanazawa, Ishikawa 920-8641, Japan
| | - Akihiko Takeuchi
- Department of Orthopedic Surgery, Graduate School of Medical Sciences, Kanazawa, Ishikawa 920-8641, Japan
| | - Shinji Miwa
- Department of Orthopedic Surgery, Graduate School of Medical Sciences, Kanazawa, Ishikawa 920-8641, Japan
| | - Kentaro Igarashi
- Department of Orthopedic Surgery, Graduate School of Medical Sciences, Kanazawa, Ishikawa 920-8641, Japan
| | - Yuta Taniguchi
- Department of Orthopedic Surgery, Graduate School of Medical Sciences, Kanazawa, Ishikawa 920-8641, Japan
| | - Hirotaka Yonezawa
- Department of Orthopedic Surgery, Graduate School of Medical Sciences, Kanazawa, Ishikawa 920-8641, Japan
| | - Sei Morinaga
- Department of Orthopedic Surgery, Graduate School of Medical Sciences, Kanazawa, Ishikawa 920-8641, Japan
| | - Takayuki Nojima
- Department of Orthopedic Surgery, Graduate School of Medical Sciences, Kanazawa, Ishikawa 920-8641, Japan.,Department of Pathology, Kanazawa University, Kanazawa, Ishikawa 920-8641, Japan
| | - Hiroyuki Tsuchiya
- Department of Orthopedic Surgery, Graduate School of Medical Sciences, Kanazawa, Ishikawa 920-8641, Japan
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20
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Sung IH, Son HJ, Park JS, Song YS, Park KC. Extraskeletal osteosarcoma misdiagnosed as heterotopic ossification after periprosthetic femoral fracture: A case report. ACTA ORTHOPAEDICA ET TRAUMATOLOGICA TURCICA 2020; 54:118-123. [PMID: 32175906 DOI: 10.5152/j.aott.2020.01.444] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/22/2022]
Abstract
Extraskeletal osteosarcoma is a malignant tumor of soft tissue characterized by osteoid production and has a very low prevalence, comprising approximately 4% of all osteosarcomas and about 1% of all soft tissue sarcomas, and a total of about 350 cases have been reported until now. Heterotopic ossification is a pathological finding of bony tissue in soft tissue regions such as muscle, skin and subcutaneous tissue. We report a case of an 86-year-old woman with a history of total hip arthroplasty (THA), in which open reduction and internal fixation were done for periprosthetic femoral Fracture. The ossified lesion misdiagnosed as heterotopic ossification initially was diagnosed as extraskeletal osteosarcoma at 6 months after the surgery. Both extraskeletal osteosarcoma and heterotopic ossification have no definite symptoms, but show radiopaque shadows on simple radiograph. Therefore, careful attention and thorough evaluation with multiple imaging tests may be necessary for the differential diagnosis of these entities.
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Affiliation(s)
- Il-Hoon Sung
- Department of Orthopedic Surgery, Hanyang University, College of Medicine, Seoul Hospital, Seoul, Korea
| | - Hee-Jung Son
- Department of Orthopedic Surgery, Hanyang University, College of Medicine, Guri Hospital, Gyeonggi, Korea
| | - Jin-Sung Park
- Department of Orthopedic Surgery, Hanyang University, College of Medicine, Guri Hospital, Gyeonggi, Korea
| | - Young-Sik Song
- Department of Orthopedic Surgery, Hanyang University, College of Medicine, Guri Hospital, Gyeonggi, Korea
| | - Ki-Chul Park
- Department of Orthopedic Surgery, Hanyang University, College of Medicine, Guri Hospital, Gyeonggi, Korea
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21
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The role of chemotherapy and radiotherapy in localized extraskeletal osteosarcoma. Eur J Cancer 2019; 125:130-141. [PMID: 31806415 DOI: 10.1016/j.ejca.2019.07.029] [Citation(s) in RCA: 51] [Impact Index Per Article: 8.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/14/2019] [Revised: 07/05/2019] [Accepted: 07/10/2019] [Indexed: 11/24/2022]
Abstract
PURPOSE The role of chemotherapy (CT) and radiotherapy (RT) for management of extraskeletal osteosarcoma (ESOS) remains controversial. We examined disease outcomes for ESOS patients and investigated the association between CT/RT with recurrence and survival. PATIENTS AND METHODS Retrospective review at 25 international sarcoma centers identified patients ≥18 years old treated for ESOS from 1971 to 2016. Patient/tumour characteristics, treatment, local/systemic recurrence, and survival data were collected. Kaplan-Meier survival and Cox proportional-hazards regression and cumulative incidence competing risks analysis were performed. RESULTS 370 patients with localized ESOS treated definitively with surgery presented with mainly deep tumours (n = 294, 80%). 122 patients underwent surgical resection alone, 96 (26%) also received CT, 70 (19%) RT and 82 (22%) both adjuvants. Five-year survival for patients with localized ESOS was 56% (95% CI 51%-62%). Almost half of patients (n = 173, 47%) developed recurrence: local 9% (35/370), distant 28% (102/370) or both 10% (36/370). Considering death as a competing event, there was no significant difference in cumulative incidence of local or systemic recurrence between patients who received CT, RT, both or neither (local p = 0.50, systemic p = 0.69). Multiple regression Cox analysis showed a significant association between RT and decreased local recurrence (HR 0.46 [95% CI 0.26-0.80], p = 0.01). CONCLUSION Although the use of RT significantly decreased local recurrences, CT did not decrease the risk of systemic recurrence, and neither CT, nor RT nor both were associated with improved survival in patients with localized ESOS. Our results do not support the use of CT; however, adjuvant RT demonstrates benefit in patients with locally resectable ESOS.
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22
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Kito F, Oyama R, Noguchi R, Hattori E, Sakumoto M, Endo M, Kobayashi E, Yoshida A, Kawai A, Kondo T. Establishment and characterization of novel patient-derived extraskeletal osteosarcoma cell line NCC-ESOS1-C1. Hum Cell 2019; 33:283-290. [PMID: 31625124 DOI: 10.1007/s13577-019-00291-z] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/03/2019] [Accepted: 10/11/2019] [Indexed: 01/22/2023]
Abstract
Extraskeletal osteosarcoma (ESOS) is a rare mesenchymal malignancy producing osteoid and bone in soft tissue without skeletal attachment. ESOS exhibits chemoresistance and poor prognosis, and is distinct from osseous osteosarcoma. The biological characteristics of ESOS are not fully understood, and patient-derived cell lines of ESOS are not available from public cell banks. Here, we established a novel cell line of ESOS and characterized its genetic and biological characteristics as well as examined its response to anti-cancer reagents. The cell line was established using tumor tissue from a 58-year-old female patient with ESOS, and named as NCC-ESOS1-C1. Phenotypes relevant to malignancy such as proliferation and invasion were examined in vitro, and genetic features were evaluated using the NCC Oncopanel assay. The response to inhibitors was monitored by screening of an anti-cancer reagent library. The cells constantly proliferated, showing spheroid formation and invasion capabilities. The NCC Oncopanel revealed the presence of actionable mutations in PIK3CA. Library screening revealed the presence of anti-cancer reagents with significant anti-proliferative effects on NCC-ESOS1-C1 at a low concentration. In conclusion, we established and characterized a novel ESOS cell line, NCC-ESOS1-C1. This cell line will be a useful resource for basic research and preclinical studies.
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Affiliation(s)
- Fumiko Kito
- Department of Innovative Seeds Evaluation, National Cancer Center Research Institute, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan
| | - Rieko Oyama
- Department of Innovative Seeds Evaluation, National Cancer Center Research Institute, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan
| | - Rei Noguchi
- Division of Rare Cancer Research, National Cancer Center Research Institute, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan
| | - Emi Hattori
- Division of Rare Cancer Research, National Cancer Center Research Institute, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan
| | - Marimu Sakumoto
- Division of Rare Cancer Research, National Cancer Center Research Institute, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan
| | - Makoto Endo
- Division of Musculoskeletal Oncology, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan
| | - Eisuke Kobayashi
- Division of Musculoskeletal Oncology, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan
| | - Akihiko Yoshida
- Department of Diagnosis Pathology, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan
| | - Akira Kawai
- Division of Musculoskeletal Oncology, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan
| | - Tadashi Kondo
- Department of Innovative Seeds Evaluation, National Cancer Center Research Institute, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan.
- Division of Rare Cancer Research, National Cancer Center Research Institute, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan.
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23
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Abstract
OBJECTIVES Extraskeletal osteosarcoma (EO) is a malignant neoplasm that produces osteoid, bone, and chondroid material without direct attachment to bone or periosteum. Surgical resection is the mainstay of treatment; the role of chemotherapy is not well defined. Therefore, we evaluated the impact of chemotherapy in the survival of patients with EO. METHODS All EO patients seen at Mayo Clinic between 1990 and 2014 were assessed. Forty-three patients were included after all archived pathology slides were reviewed to confirm the diagnosis of EO. RESULTS Of 43 patients, 37 patients had localized disease and 6 patients had metastatic disease at diagnosis. Chemotherapy was used in 73% and 75% of patients, respectively. Chemotherapy was predominantly anthracycline based, and included platinum in 22 patients (84%).Median overall survival (OS) and progression-free survival (PFS) were 50 months (95% confidence interval, 25-99), and 21 months (95% confidence interval, 13-not reached), respectively. There was a trend towards longer OS and PFS in patients who received chemotherapy. Those who received platinum-based therapy had remarkably prolonged OS (median, 182 vs. 18 mo; 5-year, 61% vs. 0%; P=0.01) and PFS (median, not reached vs. 10 mo; 5-year, 56% vs. 0%; P=0.005). Baseline characteristics were similar in the platinum and nonplatinum group.In patients who received chemotherapy, relapse/recurrence rate was lower in the platinum-based group (41%) as opposed to the nonplatinum-based group (100%; P=0.02). In the neoadjuvant setting, the overall response rate of platinum-containing regimens was 27%. CONCLUSIONS Our results suggest a clinical benefit when platinum-based chemotherapy is incorporated in the management of patients with EO. We plan to validate this further with an expanded multicenter analysis.
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24
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Wakamatsu T, Kakunaga S, Takenaka S, Outani H, Hamada K, Imura Y, Hori Y, Naka N, Kudawara I, Yoshikawa H, Ueda T. Prognostic implication of adjuvant/neoadjuvant chemotherapy consisting of doxorubicin and ifosfamide in patients with extraskeletal osteosarcoma. Int J Clin Oncol 2019; 24:1311-1319. [PMID: 31197555 DOI: 10.1007/s10147-019-01475-1] [Citation(s) in RCA: 14] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/01/2019] [Accepted: 05/21/2019] [Indexed: 11/28/2022]
Abstract
BACKGROUND Extraskeletal osteosarcoma (ESOS) is an extremely rare soft tissue sarcoma. Their prognosis remains poor. Our purposes were to identify the effective chemotherapeutic regimen for ESOS. METHODS We retrospectively reviewed 16 patients with ESOS treated at the Osaka University Orthopaedic Oncology Group between 1992 and 2012. We extracted the clinical data on patients. Kaplan-Meier method and the log-rank test were used for survival analyses. RESULTS Median age of the patients was 61.5 years (range 25-79 years). Wide local excision was performed for 11 patients and 9 patients were treated combined with chemotherapy. The 5-year disease-specific survival (DSS) rate was 53.9%. The 5-year DSS rates for patients treated with adjuvant/neoadjuvant chemotherapy or not were 66.7% or 25%, respectively (p = 0.0215). Furthermore, the 5-year DSS rates for patients treated with adjuvant/neoadjuvant chemotherapy consisting of doxorubicin and ifosfamide and those treated with other regimens were 100% or 40%, respectively (p = 0.0327). CONCLUSION The present study demonstrated that adjuvant/neoadjuvant chemotherapy, especially consisting of doxorubicin and ifosfamide, was potentially efficacious for ESOS. Further prospective study using this multimodality treatment approach to patients with ESOS should be strongly warranted.
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Affiliation(s)
- Toru Wakamatsu
- Department of Orthopaedic Surgery, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, Osaka, 565-0871, Japan.,Department of Musculoskeletal Oncology Service, Osaka International Cancer Institute, 3-1-69, Otemae, Chuo-ku, Osaka, 541-8567, Japan
| | - Shigeki Kakunaga
- Department of Orthopaedic Surgery, National Hospital Organization Osaka National Hospital, 2-1-14 Hoenzaka, Chuo-ku, Osaka, 540-0006, Japan
| | - Satoshi Takenaka
- Department of Orthopaedic Surgery, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, Osaka, 565-0871, Japan
| | - Hidetatsu Outani
- Department of Orthopaedic Surgery, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, Osaka, 565-0871, Japan
| | - Kenichiro Hamada
- Department of Orthopaedic Surgery, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, Osaka, 565-0871, Japan
| | - Yoshinori Imura
- Department of Orthopaedic Surgery, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, Osaka, 565-0871, Japan.,Department of Musculoskeletal Oncology Service, Osaka International Cancer Institute, 3-1-69, Otemae, Chuo-ku, Osaka, 541-8567, Japan
| | - Yumiko Hori
- Department of Pathology, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, Osaka, 565-0871, Japan
| | - Norifumi Naka
- Department of Orthopaedic Surgery, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, Osaka, 565-0871, Japan.,Department of Musculoskeletal Oncology Service, Osaka International Cancer Institute, 3-1-69, Otemae, Chuo-ku, Osaka, 541-8567, Japan
| | - Ikuo Kudawara
- Department of Orthopaedic Surgery, National Hospital Organization Osaka National Hospital, 2-1-14 Hoenzaka, Chuo-ku, Osaka, 540-0006, Japan
| | - Hideki Yoshikawa
- Department of Orthopaedic Surgery, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, Osaka, 565-0871, Japan
| | - Takafumi Ueda
- Department of Orthopaedic Surgery, National Hospital Organization Osaka National Hospital, 2-1-14 Hoenzaka, Chuo-ku, Osaka, 540-0006, Japan.
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Liao Z, Qiu M, Yang J, Yang Y, Zhu L, Yang B, Bai X, Xing P, Zhang J, Xing R, Teng S, Zhao J. Outcomes of surgery and/or combination chemotherapy for extraskeletal osteosarcoma: a single-center retrospective study from China. Sci Rep 2019; 9:4816. [PMID: 30886189 PMCID: PMC6423283 DOI: 10.1038/s41598-019-41089-1] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/14/2018] [Accepted: 02/28/2019] [Indexed: 11/23/2022] Open
Abstract
Extraskeletal osteosarcoma (ESOS) is an extremely rare malignancy with poor prognosis, accounting for 2-4% of all osteogenic sarcomas. The purpose of this study was to examine the oncological outcomes of this disease related to surgical treatment and/or combined adjuvant therapies and to analyze the associated prognostic factors in ESOS. From January 1990 to June 2016, 22 patients with primary ESOS were analyzed in this retrospective study. Overall survival (OS) and progression-free survival (PFS) rates were calculated by Kaplan-Meier methods and compared with log-rank test. 22 patients were diagnosed with ESOS, 19 showed localized diseases and 3 presented with metastatic lesions. The median age at diagnosis was 55.5 years. Surgery resection was performed for all patients, 18 of whom received adjuvant chemotherapy. The median follow-up time was 48.5 months. There were 10 cases of recurrence and 9 patients developed new metastases. The 5-year OS rate for all patients was 58%. For localized cohort, the 5-year OS rate was 62%, and the 3-year PFS rate was 31% with a median PFS of 16 months. Univariate analysis of related prognosis factors showed that larger size of tumor (>5.5 cm) and higher histologic grade emerged as significant factors associated with worse OS. The addition of combination chemotherapy has no effect found on OS or PFS in this study. In summary, for patients who presented with ESOS, larger tumor size and higher histologic grade indicate a lower OS rate. The combination chemotherapy does not improve the OS or PFS.
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Affiliation(s)
- Zhichao Liao
- Departments of Bone and Soft Tissue Tumor, Tianjin Medical University Cancer Institute & Hospital, Tianjin, 300060, People's Republic of China
- National Clinical Research Center of Cancer, Tianjin Medical University Cancer Institute & Hospital, Tianjin, 300060, People's Republic of China
| | - Minghan Qiu
- Department of Oncology, Tianjin Union Medical Center, Tianjin, 300121, People's Republic of China
| | - Jilong Yang
- Departments of Bone and Soft Tissue Tumor, Tianjin Medical University Cancer Institute & Hospital, Tianjin, 300060, People's Republic of China.
- National Clinical Research Center of Cancer, Tianjin Medical University Cancer Institute & Hospital, Tianjin, 300060, People's Republic of China.
| | - Yun Yang
- Departments of Bone and Soft Tissue Tumor, Tianjin Medical University Cancer Institute & Hospital, Tianjin, 300060, People's Republic of China
- National Clinical Research Center of Cancer, Tianjin Medical University Cancer Institute & Hospital, Tianjin, 300060, People's Republic of China
| | - Lei Zhu
- National Clinical Research Center of Cancer, Tianjin Medical University Cancer Institute & Hospital, Tianjin, 300060, People's Republic of China
- Departments of Molecular Imagine, Tianjin Medical University Cancer Institute & Hospital, Tianjin, 300060, People's Republic of China
| | - Bo Yang
- National Clinical Research Center of Cancer, Tianjin Medical University Cancer Institute & Hospital, Tianjin, 300060, People's Republic of China
- Departments of Pathology, Tianjin Medical University Cancer Institute & Hospital, Tianjin, 300060, People's Republic of China
| | - Xu Bai
- National Clinical Research Center of Cancer, Tianjin Medical University Cancer Institute & Hospital, Tianjin, 300060, People's Republic of China
- Departments of Radiation Oncology, Tianjin Medical University Cancer Institute & Hospital, Tianjin, 300060, People's Republic of China
| | - Peipei Xing
- Departments of Bone and Soft Tissue Tumor, Tianjin Medical University Cancer Institute & Hospital, Tianjin, 300060, People's Republic of China
- National Clinical Research Center of Cancer, Tianjin Medical University Cancer Institute & Hospital, Tianjin, 300060, People's Republic of China
| | - Jin Zhang
- Departments of Bone and Soft Tissue Tumor, Tianjin Medical University Cancer Institute & Hospital, Tianjin, 300060, People's Republic of China
- National Clinical Research Center of Cancer, Tianjin Medical University Cancer Institute & Hospital, Tianjin, 300060, People's Republic of China
| | - Ruwei Xing
- Departments of Bone and Soft Tissue Tumor, Tianjin Medical University Cancer Institute & Hospital, Tianjin, 300060, People's Republic of China
- National Clinical Research Center of Cancer, Tianjin Medical University Cancer Institute & Hospital, Tianjin, 300060, People's Republic of China
| | - Sheng Teng
- Departments of Bone and Soft Tissue Tumor, Tianjin Medical University Cancer Institute & Hospital, Tianjin, 300060, People's Republic of China
- National Clinical Research Center of Cancer, Tianjin Medical University Cancer Institute & Hospital, Tianjin, 300060, People's Republic of China
| | - Jun Zhao
- Departments of Bone and Soft Tissue Tumor, Tianjin Medical University Cancer Institute & Hospital, Tianjin, 300060, People's Republic of China.
- National Clinical Research Center of Cancer, Tianjin Medical University Cancer Institute & Hospital, Tianjin, 300060, People's Republic of China.
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26
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Extraskeletal Osteosarcomas: A Case Made for Combined Modality Local Therapy With Radiation and Surgery. Am J Clin Oncol 2019; 42:238-242. [PMID: 30614820 DOI: 10.1097/coc.0000000000000515] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/03/2023]
Abstract
OBJECTIVE We evaluated our experience treating patients with extraskeletal osteosarcoma (ESOS) using combined modality local therapy, including surgery and radiation therapy (RT), to investigate local control (LC) and other survival endpoints. METHODS We reviewed the records of 21 consecutive patients with nonmetastatic, ESOS treated with RT in combination with surgery at our institution from 1984 to 2015. Postoperative RT was used for 10 patients (48%) to a median dose of 60 Gy (range, 60 to 68 Gy). The other 11 patients (52%) received 50 Gy preoperatively. Seven patients (33%) were treated with neoadjuvant or adjuvant chemotherapy. Among the patients who received chemotherapy, all were treated with doxorubicin and ifosfamide for a median of 6 cycles (range, 4 to 6). RESULTS Median follow-up time was 120 months (range, 6 to 200 mo). Larger tumor size (>5 cm) was associated with chemotherapy use (P=0.046). The 5-year LC, distant metastatic free survival, and disease-specific survival rates were 93%, 53%, and 62%, respectively. Only 1 patient (5%) had a local recurrence at 22 months. Nine patients (43%) developed distant metastases at a median time of 9 months (range, 1 to 30 mo) without any significant factors for reduced distant metastatic free survival. Following disease relapse, there were 2 patients who were ultimately salvaged, both of whom were treated with chemotherapy and surgery. CONCLUSIONS RT in combination with surgery provides favorable LC for patients with ESOS. However, patients have a high rate of distant metastases with limited salvage.
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27
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Zhang JS, Wen G, Liu Y, Wu ZH, Chen GD, Wang H, Zhou TC. Extraskeletal osteosarcoma in right neck subcutaneous tissue: A case report of an extremely rare tumour. Mol Clin Oncol 2018; 9:149-154. [PMID: 30101012 PMCID: PMC6083428 DOI: 10.3892/mco.2018.1655] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/11/2017] [Accepted: 06/05/2018] [Indexed: 11/12/2022] Open
Abstract
Extraskeletal osteosarcoma (ESOS) is a rare soft-tissue sarcoma that is treated with surgical resection, chemotherapy and radiotherapy; however, as it is now considered to be radiation resistance, it is associated with conflicting management principles and poor outcomes. A multimodality approach is currently used to treat ESOS, which entails the incorporation of multidrug chemotherapy and/or radiotherapy coupled with surgery to obtain the best outcome; however, there are many factors that influence the treatment effects and clinical outcomes of ESOS. In the present study, a case of an 81-year-old man who suffered from primary ESOS in the subcutaneous tissue of the right-hand side of the neck was reported. The patient was treated several times with partial resection and once with radiotherapy, and was still living following 3 years of follow-up. Thus, the present case report demonstrated that surgical resection and postoperative radiotherapy regimens may be favourable in the short term with a disease-free survival of ~15 months; however, patients are prone to relapse.
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Affiliation(s)
- Jin-Shan Zhang
- Department of Radiation Oncology, Third Affiliated Hospital of Guangzhou Medical University, Guangzhou, Guangdong 510120, P.R. China
| | - Ge Wen
- Department of Radiation Oncology, Third Affiliated Hospital of Guangzhou Medical University, Guangzhou, Guangdong 510120, P.R. China
| | - Yuan Liu
- Department of Radiation Oncology, Cancer Center of Guangzhou Medical University, Guangzhou, Guangdong 510120, P.R. China
| | - Zhao-Hong Wu
- Department of Thoracic Surgery, Third Affiliated Hospital of Guangzhou Medical University, Guangzhou, Guangdong 510120, P.R. China
| | - Gang-Dong Chen
- Department of Thoracic Surgery, Third Affiliated Hospital of Guangzhou Medical University, Guangzhou, Guangdong 510120, P.R. China
| | - Hao Wang
- Department of Radiation Oncology, Third Affiliated Hospital of Guangzhou Medical University, Guangzhou, Guangdong 510120, P.R. China
| | - Tong-Chong Zhou
- Department of Radiation Oncology, Cancer Center of Guangzhou Medical University, Guangzhou, Guangdong 510120, P.R. China
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28
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Hamamoto T, Kono T, Furuie H, Ueda T, Takeno S, Hirakawa K, Arihiro K. Extraskeletal osteosarcoma in the parotid gland: A case report. Auris Nasus Larynx 2018; 45:644-647. [DOI: 10.1016/j.anl.2017.09.001] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/06/2017] [Revised: 08/24/2017] [Accepted: 09/05/2017] [Indexed: 11/17/2022]
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29
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Wang H, Miao R, Jacobson A, Harmon D, Choy E, Hornicek F, Raskin K, Chebib I, DeLaney TF, Chen YLE. Extraskeletal osteosarcoma: A large series treated at a single institution. Rare Tumors 2018; 10:2036361317749651. [PMID: 31508194 PMCID: PMC5811988 DOI: 10.1177/2036361317749651] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/12/2017] [Accepted: 10/06/2017] [Indexed: 01/26/2023] Open
Abstract
Purpose: This study is to present a large cohort of extraskeletal osteosarcoma (ESOS) and evaluate prognostic factors and treatment options. Methods: Medical records were reviewed retrospectively for 41 patients with extraskeletal osteosarcoma that was diagnosed by pathology, and treated at our institution between 1960 and 2016. Kaplan-Meier analysis and Cox proportional hazards regression were used to identify variables that affect survival outcomes. Results: 41 patients were identified from 952 osteosarcomas. 32 patients had non-metastatic disease. Prognostic factors were identified by univariate analysis and multi-variate analysis. Surgery (p<0.001), and surgery type (p<0.001) both were shown to significantly affect overall survival (OS). Chemotherapy and radiation therapy (RT) did not show any significant effect on OS, local recurrence, or progression free survival as a whole. However for patients who had incomplete resection with residual tumor RT improved OS (p=0.03). The survival curve for ESOS follows more closely that of non-rhabdomyosarcoma soft tissue sarcomas (NRSTS). Conclusions: ESOS is a very rare tumor. Attempt to achieve wide resection is the treatment of choice. However for patients who are not able to achieve complete resection, RT may improve OS. The behavior of ESOS more closely follows that of NRSTS than osteosarcoma of the bone.
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Affiliation(s)
- Haotong Wang
- Department of Radiation Oncology, Massachusetts General Hospital, Boston, MA, USA
| | - Ruoyu Miao
- Department of Radiation Oncology, Massachusetts General Hospital, Boston, MA, USA
| | - Alex Jacobson
- Department of Radiation Oncology, Massachusetts General Hospital, Boston, MA, USA
| | - David Harmon
- Department of Medical Oncology, Massachusetts General Hospital, Boston, MA, USA.,Harvard Medical School, Boston, MA, USA
| | - Edwin Choy
- Department of Medical Oncology, Massachusetts General Hospital, Boston, MA, USA.,Harvard Medical School, Boston, MA, USA
| | - Francis Hornicek
- Harvard Medical School, Boston, MA, USA.,Department of Orthopaedic Oncology, Massachusetts General Hospital, Boston, MA, USA
| | - Kevin Raskin
- Harvard Medical School, Boston, MA, USA.,Department of Orthopaedic Oncology, Massachusetts General Hospital, Boston, MA, USA
| | - Ivan Chebib
- Harvard Medical School, Boston, MA, USA.,Department of Pathology, Massachusetts General Hospital, Boston, MA, USA
| | - Thomas F DeLaney
- Department of Radiation Oncology, Massachusetts General Hospital, Boston, MA, USA.,Harvard Medical School, Boston, MA, USA
| | - Yen-Lin E Chen
- Department of Radiation Oncology, Massachusetts General Hospital, Boston, MA, USA.,Harvard Medical School, Boston, MA, USA
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30
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Abstract
Osteosarcoma (OS), chondrosarcoma, and chordoma are characterized by multiple challenges to the investigator, clinician, and patient. One consequence of their rarity among sarcomas, as well as their biologic and clinical heterogeneity, is that management guidelines are inadequate to inform the range of individual patient-treatment decisions from diagnosis, approaches to surgery, chemotherapy, radiotherapy, treatment of recurrence, palliative care, and quality of survivorship. Of high-grade sarcomas, OSs are among the most curable, with more than two-thirds of patients with localized disease likely to achieve long-term survival. Neoadjuvant chemotherapy comprising cisplatin, doxorubicin, and methotrexate with intercalated surgery is the standard of care for resectable OS in those younger than 40 years. Outcomes for OS presenting with unresectable metastases or recurrent disease, or in those older than 40 years are generally poor. Overall results have improved little for all patients with OS, and new treatments are needed. Surgical resection remains the cornerstone of management for chondrosarcoma and chordoma. However, the application of new biologic insights to therapeutic development indicates that improved treatments may soon be routine for patients with chondrosarcoma and chordoma for whom surgery alone is inadequate. For all these uncommon diseases, patients should be offered specialist expert care delivered by experienced multidisciplinary teams in high-volume centers.
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Affiliation(s)
- Jeremy S Whelan
- Jeremy S. Whelan, University College London Hospitals NHS Foundation Trust, London, United Kingdom; and Lara E. Davis, Knight Cancer Institute, Oregon Health & Science University, Portland, OR
| | - Lara E Davis
- Jeremy S. Whelan, University College London Hospitals NHS Foundation Trust, London, United Kingdom; and Lara E. Davis, Knight Cancer Institute, Oregon Health & Science University, Portland, OR
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Furrukh M, Qureshi A, Mamoon N, Fatima M. Anterior abdominal wall extraosseous osteosarcoma, occurring 40 years after para-aortic irradiation. BMJ Case Rep 2017. [PMID: 28645924 DOI: 10.1136/bcr-2017-219722] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
Extraskeletal osteosarcomas (OSs) are highly malignant soft tissue tumours associated with a poor prognosis. Only a few records of these rare aggressive neoplasms have been reported in the literature.We describe the case of a 49-year-old man, who presented to our tertiary care centre with a painful isolated lump around the umbilicus. After surgical biopsy, imaging and subsequent pathological analysis, the swelling was diagnosed to be a localised extraskeletal OS. He received previous radiation as treatment for testicular seminoma 40 years ago, which has been in remission ever since. He also happens to have testicular hydrocele since 10 years. He was subjected to resection and free flap reconstruction complicated by lower anterior abdominal wall haematoma and large pseudoaneurysm of the left femoral artery. Patient completed 60 Gy of adjuvant electron beam irradiation for close margins and is scheduled for adjuvant chemotherapy. We describe a brief account of his illness.
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Affiliation(s)
- Muhammad Furrukh
- Radiation Oncology, Shifa Internal Hospital, Islamabad, Pakistan
| | - Asim Qureshi
- Pathology, Shifa International Hospital, Islamabad, Pakistan
| | - Nadira Mamoon
- Pathology, Shifa International Hospital, Islamabad, Pakistan
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Diamantis A, Christodoulidis G, Vasdeki D, Karasavvidou F, Margonis E, Tepetes K. Giant abdominal osteosarcoma causing intestinal obstruction treated with resection and adjuvant chemotherapy. World J Gastrointest Surg 2017; 9:68-72. [PMID: 28289512 PMCID: PMC5329706 DOI: 10.4240/wjgs.v9.i2.68] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/03/2016] [Revised: 12/04/2016] [Accepted: 12/28/2016] [Indexed: 02/07/2023] Open
Abstract
Extraskeletal osteosarcoma (ESOS) is an uncommon tumor that accounts for 1% of all soft tissue sarcomas and 4% of all osteosarcomas. Its presentation may be atypical, while pain has been described as the most common symptom. Radiological findings include a large mass in the soft-tissues with massive calcifications, but no attachment to the adjacent bone or periosteum. We present the case of a 73-year-old gentle man who presented with a palpable, tender abdominal mass and symptoms of bowel obstruction. Computer tomography images revealed a large space-occupying heterogeneous, hyper dense soft tissue mass involving the small intestine. Explorative laparotomy revealed a large mass in the upper mesenteric root of the small intestine, measuring 22 cm × 12 cm × 10 cm in close proximity with the cecum, which was the cause of the bowel obstruction. Pathology confirmed the diagnosis of an ESOS. ESOS is an uncommon malignant soft tissue tumor with poor prognosis and a 5-year survival rate of less than 37%. Regional recurrence and distant metastasis to lungs, regional lymph nodes and liver can occur within the first three years of diagnosis in a high rate (45% and 65% respectively). Wide surgical resection of the mass followed by adjuvant chemotherapy or radiotherapy has been the treatment of choice.
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Healy C, Kahn LB, Kenan S. Subcutaneous extraskeletal osteosarcoma of the forearm: a case report and review of the literature. Skeletal Radiol 2016; 45:1307-11. [PMID: 27357312 DOI: 10.1007/s00256-016-2426-3] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/29/2016] [Revised: 06/11/2016] [Accepted: 06/16/2016] [Indexed: 02/02/2023]
Abstract
Extraskeletal osteosarcoma (ESOS) originating in the subcutaneous tissue is a rare occurrence, accounting for less than 10 % of ESOS cases. Osteosarcoma of extraskeletal origin accounts for approximately 2-4 % of all osteosarcomas, and 1 % of soft tissue sarcomas. We report a case of an 80-year-old female with an isolated primary subcutaneous tumor of the forearm. After imaging, surgical excision, and pathological analysis, the diagnosis of a subcutaneous osteosarcoma was made. This report documents the clinical and pathological findings of subcutaneous ESOS in this case, along with a review of previous cases of subcutaneous ESOS.
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Affiliation(s)
- Christopher Healy
- Department of Orthopaedic Surgery, Hofstra Northwell School of Medicine, Manhasset, NY, USA.
| | - Leonard B Kahn
- Department of Pathology, Hofstra Northwell School of Medicine, Manhasset, NY, USA
| | - Samuel Kenan
- Department of Orthopaedic Surgery, Hofstra Northwell School of Medicine, Manhasset, NY, USA
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Abstract
Objectives: To report characteristics, treatment, and outcomes for an international cohort of patients with extraskeletal osteosarcoma (ESOS). Materials and Methods: Through the Rare Cancer Network, retrospective data on patients with ESOS were collected. Patient characteristics, multimodality treatment information, and survival status were analyzed. Results: Thirty-seven patients in 4 health care institutions were identified. Thirty-one (86%) patients had grade 3 or 4 tumors. Most patients (27 [73%]) had stage III disease. Fourteen (38%) received neoadjuvant chemotherapy or chemoradiation. Of 28 (85%) who underwent surgery, 21 (75%) had free margins achieved and 15 (41%) subsequently received adjuvant chemotherapy. At median follow-up of 45 months, 20 (55%) patients were alive, 13 (43%) of whom were disease free. Univariate analysis showed that poor overall survival was related to stage IV (P<0.001), no surgery (P<0.001), primary size >10 cm (P=0.002), and age (P=0.002). In multivariate analysis, primary size >10 cm (P=0.005) was prognostic for overall survival. For patients without metastases, univariate analysis showed disease-free survival (DFS) related to primary size >10 cm (P=0.003), surgery (P=0.004), local recurrence (P=0.003), and age (P<0.001). In multivariate analysis for DFS, primary size >10 cm (P=0.01) and older age (P<0.001) were significant for worse outcome. Conclusions: Multimodality treatment remains standard for localized ESOS, with indications for neoadjuvant therapy less clear. Larger tumor size and older age were prognostic of poorer DFS.
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Tamang TGL, Shuster M, Chandra AB. Primary Hepatic Osteosarcoma: A Rare Cause of Primary Liver Tumor. CLINICAL MEDICINE INSIGHTS-CASE REPORTS 2016; 9:31-3. [PMID: 27081321 PMCID: PMC4827792 DOI: 10.4137/ccrep.s38384] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/30/2015] [Revised: 02/21/2016] [Accepted: 02/23/2016] [Indexed: 01/26/2023]
Abstract
INTRODUCTION Extraosseous osteosarcomas are rare, accounting for approximately 4% of all osteosarcomas. A literature review yields very few cases of osteosarcoma primarily arising from the hepatic parenchyma. CASE REPORT This report describes a case of a man in his 50s with a history of hepatitis C and cirrhosis who presented with 5 days of progressive right upper quadrant pain. Magnetic resonance imaging of the abdomen and pelvis demonstrated a 4.4 cm × 4.8 cm × 4.8 cm right hepatic lobe mass with a large area of necrosis and peripheral enhancement. The subsequent liver biopsy showed few cores of tumor composed of fibroblastic malignant cells producing lace-like osteoid matrix. Osteosarcomatous foci in other parts of the body were excluded by performing extensive physical examination, radiologic imaging, and biopsy. Hence, a primary osteosarcoma was diagnosed. The patient underwent portal vein embolization in preparation for a surgical resection of the right liver lobe. He was admitted six weeks after the embolization for dyspnea and abdominal distension and expired due to abdominal hematoma and pulmonary embolism. CONCLUSION Based on the rarity, lack of consensus in treatment, and dismal prognosis, extraosseous osteosarcoma should be considered a separate entity from osseous osteosarcoma. More data and research are needed in this rare and understudied malignancy.
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Affiliation(s)
| | - Marina Shuster
- Department of Internal Medicine, Maimonides Medical Center, Brooklyn, NY, USA
| | - Abhinav B Chandra
- Division of Hematology and Oncology, Maimonides Medical Center, Brooklyn, NY, USA
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Casey DL, van de Rijn M, Riley G, Tung KW, Mohler DG, Donaldson SS. Extraskeletal osteosarcoma of the hand: the role of marginal excision and adjuvant radiation therapy. Hand (N Y) 2015; 10:602-6. [PMID: 26568711 PMCID: PMC4641083 DOI: 10.1007/s11552-015-9760-0] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 10/23/2022]
Abstract
BACKGROUND Extraskeletal osteosarcoma of the hand is rare, and its optimal modality of local control is not currently known. METHODS A literature search was performed to identify studies that describe the treatment and outcomes of extraskeletal osteosarcoma. A second literature search was performed to identify studies that describe the treatment and outcomes of extraskeletal osteosarcoma of the hand specifically. RESULTS The role of adjuvant radiation for extraskeletal osteosarcoma is not well defined. All cases in the literature describing treatment of extraskeletal osteosarcoma of the hand utilized amputation, and none of the patients described received radiation therapy. However, there are multiple reports showing excellent local control, minimal toxicity, and superior functional outcome with limb conservation and radiation rather than amputation of the hand in pediatric and adult soft tissue sarcoma. CONCLUSION For extraskeletal osteosarcoma of the hand, we recommend a treatment approach with the goal of preservation of form and function using limb-sparing surgery and planned postoperative radiation.
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Affiliation(s)
- Dana L. Casey
- Department of Radiation Oncology, Stanford University, Stanford, CA USA
| | | | - Geoffrey Riley
- Department of Radiology, Stanford University, Stanford, CA USA
| | - Ka-Wah Tung
- Department of Radiology, Stanford University, Stanford, CA USA
| | - David G. Mohler
- Department of Surgery, Stanford University, Stanford, CA USA
| | - Sarah S. Donaldson
- Department of Radiation Oncology, Stanford University, Stanford, CA USA
- Department of Radiation Oncology, Stanford University Cancer Center, 875 Blake Wilbur Drive, Stanford, CA 94305 USA
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Fan Z, Patel S, Lewis VO, Guadagnolo BA, Lin PP. Should High-grade Extraosseous Osteosarcoma Be Treated With Multimodality Therapy Like Other Soft Tissue Sarcomas? Clin Orthop Relat Res 2015; 473. [PMID: 26197952 PMCID: PMC4586239 DOI: 10.1007/s11999-015-4463-y] [Citation(s) in RCA: 21] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/31/2023]
Abstract
BACKGROUND Extraosseous osteosarcoma is rare, and the most appropriate therapy is unclear because there are few studies regarding its treatment. The effectiveness of radiation and chemotherapy remains uncertain owing to conflicting results in previous reports. QUESTIONS/PURPOSES To review our experience with contemporary multimodality treatment, we asked: (1) What is the disease-specific survival and local relapse-free survival? (2) Does American Joint Commission on Cancer (AJCC) stage, tumor size, or location relate to disease outcome? (3) Does radiation therapy improve local control or survival? (4) Do doxorubicin and ifosfamide improve local control or survival? METHODS Between 1990 and 2012, we treated 40 patients for localized, high-grade extraosseous osteosarcoma. In this retrospective study, we could determine the status of 36 patients (90%) either to death or for a minimum of 24 months of followup; four (10%) were lost to followup before 24 months. There were 11 patients with AJCC Stage IIA and 25 with Stage III disease. All patients underwent wide surgical excision. Of the patients with Stage IIA disease, four received radiation and none received chemotherapy. Of the patients with Stage III disease, six received radiation, seven were treated with chemotherapy, and six received radiation and chemotherapy. During the study period, high-dose doxorubicin and ifosfamide was the preferred chemotherapy regimen for patients younger than 60 years with normal cardiac and renal function. Local relapse-free survival and disease-specific survival were determined by Kaplan-Meier analysis using a prospectively maintained institutional database supplemented by information from the institutional tumor registry. The Cox proportional hazard model was used to determine the effect of various factors on local recurrence and patient survival. RESULTS At 5 years, local relapse-free survival was 47% (95% CI, 27%-64%), and disease-specific survival was 53% (95% CI, 35%-68%). In multivariate analysis, AJCC stage, which depends on tumor size, was the strongest predictor of local relapse-free survival (hazard ratio [HR] = 0.17, p = 0.02), while tumor depth was the best predictor of disease-specific survival (HR = 5.6, p = 0.02). Radiation improved local relapse-free survival (HR = 0.30, p = 0.03) but not disease-specific survival in multivariate analysis. A regimen of doxorubicin and ifosfamide was associated with better local relapse-free survival for patients with Stage III disease (HR = 0.16, p = 0.04) but not disease-specific survival (HR = 0.32, p = 0.08). CONCLUSIONS With the limited number of patients in our study, it appears that extraosseous osteosarcoma behaves differently than osteosarcoma of bone. Multimodality treatment that includes doxorubicin and ifosfamide-based chemotherapy, radiation, and surgery may be a valid therapeutic strategy for Stage III disease, but larger, prospective studies will be needed to verify our preliminary observations. LEVEL OF EVIDENCE Level III, therapeutic study.
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Affiliation(s)
- Zhengfu Fan
- Department of Orthopaedic Oncology, Peking University Cancer Hospital & Institute, Beijing, China
| | - Shreyaskumar Patel
- Department of Sarcoma Medical Oncology, University of Texas MD Anderson Cancer Center, Houston, TX, USA
| | - Valerae O Lewis
- Department of Orthopaedic Oncology, University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Unit 1448, Houston, TX, 77030, USA
| | - B Ashleigh Guadagnolo
- Department of Radiation Oncology, University of Texas MD Anderson Cancer Center, Houston, TX, USA
| | - Patrick P Lin
- Department of Orthopaedic Oncology, University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Unit 1448, Houston, TX, 77030, USA.
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Strippoli S, Traversa M, Cramarossa A, Popescu O, Lorusso V, Guida M. Long-term response of gemcitabine plus docetaxel chemotherapy regimen for extraskeletal osteosarcoma: A case report. Oncol Lett 2015; 9:2567-2571. [PMID: 26137107 DOI: 10.3892/ol.2015.3111] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/10/2014] [Accepted: 02/05/2015] [Indexed: 11/06/2022] Open
Abstract
Extraskeletal osteosarcomas (EOSs) are rare variants of primary osteosarcoma of the bone, and are defined as sarcomas located in the soft tissues and characterized by osteoid production. EOS exhibits distinctive demographic, imaging and prognostic features compared with osteosarcoma of bone origin. The available data are contradictory with regard to the use of chemotherapy regimens in the management of EOS. The present study describes a case of EOS that progressed following two lines of therapy oriented to soft-tissue and bone sarcoma histology, respectively. As a gemcitabine-docetaxel combination schedule has demonstrated synergistic activity against bone and soft-tissue sarcoma histologies, this chemotherapy regimen was selected as salvage therapy. The treatment was well-tolerated and induced a long lasting partial response for ~14 months. To the best of our knowledge, this is the first report involving the clinical use of this combination regimen for the treatment of EOS. Furthermore, as demonstrated in this report, EOS may maintain relative chemosensitivity, indicating the potential to control advanced disease in the long term and to plan subsequent chemotherapy regimens.
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Affiliation(s)
- Sabino Strippoli
- Department of Medical Oncology, National Cancer Research Centre 'Giovanni Paolo II', Bari 70124, Italy
| | - Michele Traversa
- Department of Radiology, National Cancer Research Centre 'Giovanni Paolo II', Bari 70124, Italy
| | - Antonio Cramarossa
- Department of Radiology, National Cancer Research Centre 'Giovanni Paolo II', Bari 70124, Italy
| | - Ondina Popescu
- Department of Histopathology Unit, National Cancer Research Centre 'Giovanni Paolo II', Bari 70124, Italy
| | - Vito Lorusso
- Department of Medical Oncology, National Cancer Research Centre 'Giovanni Paolo II', Bari 70124, Italy
| | - Michele Guida
- Department of Medical Oncology, National Cancer Research Centre 'Giovanni Paolo II', Bari 70124, Italy
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Duffy D, Selmic LE, Kendall AR, Powers BE. Outcome following treatment of soft tissue and visceral extraskeletal osteosarcoma in 33 dogs: 2008-2013. Vet Comp Oncol 2015; 15:46-54. [PMID: 25643914 DOI: 10.1111/vco.12141] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/13/2014] [Revised: 12/22/2014] [Accepted: 12/22/2014] [Indexed: 11/26/2022]
Abstract
Extraskeletal osteosarcoma (EOS) is a rare, highly malignant mesenchymal neoplasm arising from viscera or soft tissues characterised by the formation of osteoid in the absence of bone involvement. Owing to the rarity of these neoplasms very little information exists on treatment outcomes. The purpose of this study was to describe the outcome following surgical treatment of non-mammary and non-thyroidal soft tissue and visceral EOS in dogs. Thirty-three dogs were identified; the most common primary tumour site was the spleen. Dogs that had wide or radical tumour excision had longer survival times compared with dogs that had only marginal tumour excision performed [median survival time of 90 days (range: 0-458 days) versus median survival time of 13 days (range: 0-20 days)]. The use of surgery should be considered in the management of dogs with non-mammary and non-thyroidal soft tissue and visceral EOS.
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Affiliation(s)
- D Duffy
- Veterinary Clinical Sciences, Purdue Veterinary Teaching Hospital, West LaFayette, IN, USA
| | - L E Selmic
- Department of Veterinary Clinical Medicine, University of Illinois, Urbana, IL, USA
| | - A R Kendall
- Veterinary Clinical Sciences, Purdue Veterinary Teaching Hospital, West LaFayette, IN, USA
| | - B E Powers
- Colorado State University Diagnostic Laboratories, Fort Collins, CO, USA
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Bhatt NR, Kakked GA, Merchant R, Bhatt R. Extraskeletal osteosarcoma of the larynx: an extremely unusual tumour. BMJ Case Rep 2014; 2014:bcr-2014-206759. [PMID: 25323286 DOI: 10.1136/bcr-2014-206759] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
Osteosarcoma of the larynx is probably the rarest mesenchymal tumour of the larynx, with only 16 cases reported so far. The majority of them occur in males between the sixth and eighth decades of life. Patients usually present with non-specific symptoms such as dysphonia and upper airway compromise. The most common site of distant metastasis is the lung. Clinically, the tumour follows an aggressive course and is associated with high mortality. The case we present is unusual as it occurred at a young age (38 years) as compared with the norm and it did not arise from the endolarynx, unlike many of the other cases. This was the only known case where a Pearson near-total laryngectomy was performed whereby the patient's natural speech mechanism was preserved. This surgery was possible because the contralateral half of the larynx was clearly disease free and the interarytenoid region was uninvolved. The patient underwent postoperative adjuvant external beam radiotherapy beginning 4 weeks after surgery. The patient is doing well after 15 months of follow-up and shows no signs of recurrence.
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Affiliation(s)
- Nikita R Bhatt
- Department of Surgery, SSG Hospital, Baroda, Gujarat, India
| | | | - Rajiv Merchant
- Department of Orthopedics, Vincent's University Hospital, Dublin, Ireland
| | - Rajiv Bhatt
- Department of Oncosurgery, Shubhechha Multispecialty Hospital, Vadodara, Gujarat, India
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Clinical Features and Outcomes Differ between Skeletal and Extraskeletal Osteosarcoma. Sarcoma 2014; 2014:902620. [PMID: 25294959 PMCID: PMC4175789 DOI: 10.1155/2014/902620] [Citation(s) in RCA: 31] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/23/2014] [Revised: 08/21/2014] [Accepted: 08/26/2014] [Indexed: 02/06/2023] Open
Abstract
Background. Extraskeletal osteosarcoma (ESOS) is a rare subtype of osteosarcoma. We investigated patient characteristics, overall survival, and prognostic factors in ESOS. Methods. We identified cases of high-grade osteosarcoma with known tissue of origin in the Surveillance, Epidemiology, and End Results database from 1973 to 2009. Demographics were compared using univariate tests. Overall survival was compared with log-rank tests and multivariate analysis using Cox proportional hazards methods. Results. 256/4,173 (6%) patients with high-grade osteosarcoma had ESOS. Patients with ESOS were older, were more likely to have an axial tumor and regional lymph node involvement, and were female. Multivariate analysis showed ESOS to be favorable after controlling for stage, age, tumor site, gender, and year of diagnosis [hazard ratio 0.75 (95% CI 0.62 to 0.90); p = 0.002]. There was an interaction between age and tissue of origin such that older patients with ESOS had superior outcomes compared to older patients with skeletal osteosarcoma. Adverse prognostic factors in ESOS included metastatic disease, larger tumor size, older age, and axial tumor site. Conclusion. Patients with ESOS have distinct clinical features but similar prognostic factors compared to skeletal osteosarcoma. Older patients with ESOS have superior outcomes compared to older patients with skeletal osteosarcoma.
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Almadani N, Alsaad KO, Al-Matrafi H, Al Hadab A, Abdullah N, AlKushi A. Urinary bladder radiotherapy-related chondroblastic osteosarcoma: Rare case report and review of literature. Urol Ann 2014; 6:247-50. [PMID: 25125901 PMCID: PMC4127865 DOI: 10.4103/0974-7796.134289] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/08/2012] [Accepted: 02/10/2013] [Indexed: 11/04/2022] Open
Abstract
Radiation-related osteosarcomas are well described malignant mesenchymal neoplasms, yet their pathogenesis is not fully understood. They are generally classified into either skeletal osteosarcomas, or their and rare soft tissue counterpart. The occurrence of osteosarcoma in the urinary bladder (UB) following radiotherapy is exceedingly rare. To the best of our knowledge, only two cases of radiation-related urinary bladder osteosarcoma have been published; we herein describe another case of an 85-year-old man who developed post radiotherapy chondroblastic osteosarcoma of the urinary bladder four years following initial surgical resection and radiotherapy for bladder urothelial carcinoma. We believe that this is the first case of radiation-related chondroblastic osteosarcoma arising in the urinary bladder. In addition, we review the literature and explore the possible histogenesis of this rare neoplasm.
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Affiliation(s)
- Noorah Almadani
- Department of Pathology and Laboratory Medicine, King Abdulaziz Medical City and College of Medicine, King Saud University for Health Sciences, Riyadh, Saudi Arabia
| | - Khaled O Alsaad
- Department of Pathology and Laboratory Medicine, King Abdulaziz Medical City and College of Medicine, King Saud University for Health Sciences, Riyadh, Saudi Arabia
| | - Hamoud Al-Matrafi
- Department of Surgery, Section of Urology, King Abdulaziz Medical City and College of Medicine, King Saud University for Health Sciences, Riyadh, Saudi Arabia
| | - Abdulrahman Al Hadab
- Department of Radiation Oncology, King Abdulaziz Medical City and College of Medicine, King Saud University for Health Sciences, Riyadh, Saudi Arabia
| | - Nouf Abdullah
- Department of Diagnostic Imaging, King Abdulaziz Medical City and College of Medicine, King Saud University for Health Sciences, Riyadh, Saudi Arabia
| | - Abdulmohsen AlKushi
- Department of Pathology and Laboratory Medicine, King Abdulaziz Medical City and College of Medicine, King Saud University for Health Sciences, Riyadh, Saudi Arabia
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Choi LE, Healey JH, Kuk D, Brennan MF. Analysis of outcomes in extraskeletal osteosarcoma: a review of fifty-three cases. J Bone Joint Surg Am 2014; 96:e2. [PMID: 24382730 PMCID: PMC6948789 DOI: 10.2106/jbjs.m.00339] [Citation(s) in RCA: 42] [Impact Index Per Article: 3.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/01/2023]
Abstract
BACKGROUND Extraskeletal osteosarcoma is a rare soft-tissue sarcoma about which little is known. The objectives of this study were to describe the clinical features and natural history of extraskeletal osteosarcoma and to investigate factors affecting outcomes. METHODS A retrospective review of a prospectively maintained database of patients diagnosed with soft-tissue sarcoma was conducted. Patients with pathologically confirmed extraskeletal osteosarcoma from 1982 to 2012 were identified and were included in the analysis. Medical records were reviewed for clinical features, treatment, and outcomes. RESULTS Fifty-three patients were identified from the database: forty-two presented with localized disease, two presented with metastatic disease, and nine presented with recurrent (local and/or distant) disease. The median patient age at diagnosis was sixty-four years, with a median follow-up time of thirty-four months (range, one to 290 months) for survivors. Of the fifty-three patients who were identified, forty-one had lesions in the extremities, fifty-one had high-grade lesions, forty had lesions >5 cm, and forty-two had deep lesions. For patients presenting with localized disease, the median survival was 45.8 months with a three-year cumulative incidence of death due to disease of 39%. All patients with localized disease were managed with surgical resection of the primary tumor: nineteen with surgery only, ten with adjuvant radiation, five with adjuvant chemotherapy, and eight with both radiation and chemotherapy. Eighteen patients relapsed: two patients had local recurrences, ten patients had distant metastases, and six patients had local recurrences and distant metastases. In log-rank analysis, patients with superficial tumors and negative margins at resection had a higher three-year event-free survival. No significant association of disease-specific or event-free survival was found with the addition of radiation, chemotherapy, or both to surgery. CONCLUSIONS For patients presenting with localized extraskeletal osteosarcoma, three-year event-free survival was higher for patients with superficial tumors and negative margins at resection. Radiation and chemotherapeutic treatment were not associated with a lower incidence of death due to disease or a longer event-free survival.
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Affiliation(s)
- Lisa E. Choi
- Orthopaedic Surgery Service (L.E.C. and J.H.H.), Gastric and Mixed Tumor Service (M.F.B.), Department of Surgery, and Department of Epidemiology and Biostatistics (D.K.), Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, NY 10065. E-mail address for J.H. Healey:
| | - John H. Healey
- Orthopaedic Surgery Service (L.E.C. and J.H.H.), Gastric and Mixed Tumor Service (M.F.B.), Department of Surgery, and Department of Epidemiology and Biostatistics (D.K.), Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, NY 10065. E-mail address for J.H. Healey:
| | - Deborah Kuk
- Orthopaedic Surgery Service (L.E.C. and J.H.H.), Gastric and Mixed Tumor Service (M.F.B.), Department of Surgery, and Department of Epidemiology and Biostatistics (D.K.), Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, NY 10065. E-mail address for J.H. Healey:
| | - Murray F. Brennan
- Orthopaedic Surgery Service (L.E.C. and J.H.H.), Gastric and Mixed Tumor Service (M.F.B.), Department of Surgery, and Department of Epidemiology and Biostatistics (D.K.), Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, NY 10065. E-mail address for J.H. Healey:
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Wu Z, Chu X, Meng X, Xu C. An abdominal extraskeletal osteosarcoma: A case report. Oncol Lett 2013; 6:990-992. [PMID: 24137451 PMCID: PMC3796392 DOI: 10.3892/ol.2013.1517] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/11/2013] [Accepted: 07/19/2013] [Indexed: 11/06/2022] Open
Abstract
Primary abdominal extraskeletal osteosarcoma (EOS) is a rare carcinoma. The present study reports a case of a primary abdominal EOS involving the greater omentum and also presents a review of the literature on the etiology, diagnosis, differential diagnosis, pathological features, treatment and prognosis of the disease. The patient in the present study underwent laparoscopic surgery. A pathological examination revealed that the tumor tissues contained malignant and primitive spindle cells with varying amounts of neoplastic osteoid and osseous or cartilaginous tissue. The post-operative follow-up appointments were scheduled at three-month intervals for two years. The tumor recurred three months after the surgery.
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Affiliation(s)
- Zhiming Wu
- Department of General Surgery, Shaoxing Hospital, China Medical University, Shaoxing, Zhejiang 312030, P.R. China
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Hoch M, Ali S, Agrawal S, Wang C, Khurana JS. Extraskeletal osteosarcoma: a case report and review of the literature. J Radiol Case Rep 2013; 7:15-23. [PMID: 24421944 DOI: 10.3941/jrcr.v7i7.1245] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022] Open
Abstract
We report an instructive case of extraskeletal osteosarcoma in a 63-year-old African American male who presented after an episode of recent trauma, with clinical and radiological features characteristic of this neoplasm. Osteosarcoma is the most common primary malignant tumor of bone in young adults, but the extraskeletal variety is very uncommon. The radiological and pathological features of this neoplasm will be discussed, along with a review of the literature.
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Affiliation(s)
- Michael Hoch
- Department of Radiology, Temple University Hospital, Philadelphia, USA
| | - Sayed Ali
- Department of Radiology, Temple University Hospital, Philadelphia, USA
| | - Shefali Agrawal
- Department of Surgery, Temple University Hospital, Philadelphia, USA
| | - Congli Wang
- Department of Pathology, Temple University Hospital, Philadelphia, USA
| | - Jasvir S Khurana
- Department of Pathology, Temple University Hospital, Philadelphia, USA
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Bellan DG, Jesus-Garcia R, Teresa de Seixas Alves M, Petrilli MDT, Petrilli AS, Korukian M, Carai Maia Viola D, Schoedl MF, Senerchia AA. Extraskeletal Osteosarcoma of the Hand in an Adolescent: A Case Report. JBJS Case Connect 2013; 3:e6. [PMID: 29252311 DOI: 10.2106/jbjs.cc.l.00004] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/14/2022]
Affiliation(s)
- Davi Gabriel Bellan
- Department of Ortopedia, Universidade Federal de São Paulo, Rua Napoleão de Barros, 715 CEP 04038-032, São Paulo/SP, Brazil.
| | - Reynaldo Jesus-Garcia
- Department of Ortopedia, Universidade Federal de São Paulo, Rua Napoleão de Barros, 715 CEP 04038-032, São Paulo/SP, Brazil.
| | - Maria Teresa de Seixas Alves
- Department of Patologia, Universidade Federal de São Paulo, Rua Botucatu, 740 CEP 04023-900, São Paulo/SP, Brazil
| | - Marcelo de Toledo Petrilli
- Department of Ortopedia, Universidade Federal de São Paulo, Rua Napoleão de Barros, 715 CEP 04038-032, São Paulo/SP, Brazil.
| | - Antonio Sérgio Petrilli
- IOP/GRAACC - Department of Pediatria, Universidade Federal de São Paulo, Rua Botucatu, 743 CEP 04023-062, São Paulo/SP, Brazil
| | - Marcos Korukian
- Department of Ortopedia, Universidade Federal de São Paulo, Rua Napoleão de Barros, 715 CEP 04038-032, São Paulo/SP, Brazil.
| | - Dan Carai Maia Viola
- Department of Ortopedia, Universidade Federal de São Paulo, Rua Napoleão de Barros, 715 CEP 04038-032, São Paulo/SP, Brazil.
| | - Murillo Ferri Schoedl
- Department of Ortopedia, Universidade Federal de São Paulo, Rua Napoleão de Barros, 715 CEP 04038-032, São Paulo/SP, Brazil.
| | - Andreza Almeida Senerchia
- IOP/GRAACC - Department of Pediatria, Universidade Federal de São Paulo, Rua Botucatu, 743 CEP 04023-062, São Paulo/SP, Brazil
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Rao UNM, Hood BL, Jones-Laughner JM, Sun M, Conrads TP. Distinct profiles of oxidative stress-related and matrix proteins in adult bone and soft tissue osteosarcoma and desmoid tumors: a proteomics study. Hum Pathol 2012; 44:725-33. [PMID: 23063503 DOI: 10.1016/j.humpath.2012.06.023] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/06/2012] [Revised: 06/12/2012] [Accepted: 06/15/2012] [Indexed: 12/20/2022]
Abstract
Osteosarcomas rarely occur in older adults. Proteomics has not been reported to date in osteosarcoma occurring in the older adult population. This proteomic investigation was conducted to identify differentially expressed proteins in osteosarcoma occurring in various backgrounds from older adults. Desmoid tumors, known to recur locally but not metastasize, were also analyzed. Protein digests isolated from formalin-fixed, paraffin-embedded tumor tissue specimen representing 14 primary osteosarcomas of soft tissue and bone and 18 desmoid tumors were analyzed by high-resolution liquid chromatography-tandem mass spectrometry for protein identification and relative quantification by spectral counting. Elevated abundance levels of several proteins including heat shock protein 90 (HSP90), elastin microfibril interface-located protein 1, and clusterin were identified in osteosarcoma with slight differences in proteomic profiles. Desmoids had an abundance of collagen II and periostin only. The findings were confirmed by immunohistochemical staining for HSP90 and clusterin in the experimental samples and additionally in 16 posttherapy conventional osteosarcomas in tissue microarrays constructed from heterogeneous sarcomas and benign lesions. All osteosarcomas were positive for HSP90 and clusterin to a variable extent. One case of well-differentiated parosteal osteosarcoma was negative. Thirty of 75 other high-grade sarcomas including cases of chondrosarcoma were positive for HSP90. Low-grade and benign lesions and scars and 18 desmoid tumors had little or no expression of these proteins. HSP90 and clusterin represent candidate markers of aggressiveness in osteosarcoma occurring in older adults and may be indicative of drug resistance.
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Affiliation(s)
- Uma N M Rao
- Department of Pathology, University of Pittsburgh, University of Pittsburgh Medical Center Presbyterian/Shadyside Hospitals, Pittsburgh, PA 15232, USA.
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Abstract
Extraskeletal osteosarcoma (ESOS) is a rare malignancy, usually arising in older adults. We were unable to find reports of children or adolescents affected by an ESOS of the breast. Here, we present the case of a high-grade osteosarcoma arising in the breast of a 16-year-old girl. The tumor was treated with breast-conserving resections and adjuvant multiagent chemotherapy, based on a regimen of doxorubicin, high-dose methotrexate, cisplatin, and ifosfamide. At last follow-up, the patient was in first complete remission, 29 months after initial diagnosis.
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Cates JMM, Coffin CM. Extraskeletal cartilaginous, osseous, and chordoid tumors in children and adolescents. Pediatr Dev Pathol 2012; 15:255-66. [PMID: 22420731 DOI: 10.2350/10-07-0875-pb.1] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/02/2023]
Abstract
Extraskeletal cartilaginous, osseous, and chordoid tumors are extraordinarily rare in children and adolescents. These lesions are diagnostically challenging due to their rarity and their overlap with metastatic osteosarcoma, reactive or metabolic calcifying and bone-forming masses, various pseudosarcomatous proliferations such as myositis ossificans, and other rare genetic or metabolic disorders. This article reviews the clinicopathologic features and differential diagnosis of extraskeletal cartilaginous, osseous, and chordoid neoplasms in the first two decades of life and highlights the use of diagnostic adjuncts.
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Affiliation(s)
- Justin M M Cates
- Department of Pathology, Microbiology, and Immunology, Vanderbilt University, Nashville, TN, USA
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