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Yu PP, Liu XC, Yin L, Yin G. Aggressive fibromatosis of the sigmoid colon: A case report. World J Gastrointest Oncol 2024; 16:3716-3722. [PMID: 39171176 PMCID: PMC11334028 DOI: 10.4251/wjgo.v16.i8.3716] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/13/2024] [Revised: 06/08/2024] [Accepted: 07/02/2024] [Indexed: 08/07/2024] Open
Abstract
BACKGROUND Aggressive fibromatosis (AF), also known as desmoid tumor or desmoid-type fibromatosis, is a rare soft tissue neoplasm that can occur in almost any part of the body. Although it is a benign disease, AF is aggressive and infiltrative and has a high recurrence rate after surgery. Common sites for intra-abdominal AF are the small bowel mesentery, retroperitoneum, and pelvis. AF in the colon is extremely rare. CASE SUMMARY Here, we report the first case of sigmoid colon AF, which was accidentally discovered in a 27-year-old woman during laparoscopic myomectomy. Computed tomography confirmed a slightly enhanced mass in the sigmoid colon. Subsequent colonoscopy did not reveal a mass in the colonic lumen, but a suspected external compress was found in the sigmoid colon. Surgical disease involving a gastrointestinal stromal tumor was suspected. The patient underwent laparoscopic exploration, and sigmoidectomy with a negative margin was performed to excise the mass. Postoperative immunohistochemistry revealed that the mass was an AF. The patient recovered well and was recurrence-free at the 30-month follow-up without adjuvant therapy. CONCLUSION AF should be considered in the differential diagnosis of subepithelial colon masses. Radical resection alone can achieve good outcomes.
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Affiliation(s)
- Pan-Pan Yu
- Department of Gastrointestinal and Anal Surgery, The Affiliated Hangzhou First People's Hospital, School of Medicine, Westlake University, Hangzhou 310006, Zhejiang Province, China
- Department of Gastric Surgery, Zhejiang Cancer Hospital, Hangzhou 310022, Zhejiang Province, China
| | - Xin-Chun Liu
- Department of Gastrointestinal and Anal Surgery, The Affiliated Hangzhou First People's Hospital, School of Medicine, Westlake University, Hangzhou 310006, Zhejiang Province, China
| | - Lu Yin
- Department of Pathology, The Affiliated Hangzhou First People's Hospital, School of Medicine, Westlake University, Hangzhou 310006, Zhejiang Province, China
| | - Guang Yin
- Department of Gastrointestinal and Anal Surgery, The Affiliated Hangzhou First People's Hospital, School of Medicine, Westlake University, Hangzhou 310006, Zhejiang Province, China
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Foster CR, Strauss M, Hornick JL, Habeeb O. Desmoid Fibromatosis With TP53 Mutation and Striking Nuclear Pleomorphism. Int J Surg Pathol 2023; 31:1565-1571. [PMID: 36908221 DOI: 10.1177/10668969231160255] [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] [Indexed: 03/14/2023]
Abstract
Desmoid fibromatosis is a myofibroblastic neoplasm of intermediate biologic potential, which has a strong predilection for local recurrence but does not metastasize. Arranged in long, sweeping fascicles with infiltrative borders, desmoid fibromatosis typically consists of uniform, bland myofibroblastic spindle cells that harbor mutation of CTNNB1 (or less often APC). In this report, we present a remarkable case of desmoid fibromatosis associated with striking nuclear pleomorphism. We hypothesize that this striking pleomorphism is due to a germline TP53 mutation, a finding first suspected due to strong and diffuse p53 staining by immunohistochemistry and subsequently confirmed by molecular testing. The combination of the pleomorphism and TP53 mutation in desmoid fibromatosis represents a novel finding unreported in the literature.
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Affiliation(s)
- Charlotte R Foster
- Histopathology Department, LabPlus, Auckland City Hospital, Auckland, New Zealand
| | - Magreet Strauss
- Histopathology Department, LabPlus, Auckland City Hospital, Auckland, New Zealand
| | | | - Omar Habeeb
- Histopathology Department, Middlemore Hospital, Auckland, New Zealand
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Desmoid tumor of trapezius muscle: A case report. Ann Med Surg (Lond) 2021; 72:103127. [PMID: 34925822 PMCID: PMC8649079 DOI: 10.1016/j.amsu.2021.103127] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/26/2021] [Revised: 11/22/2021] [Accepted: 11/30/2021] [Indexed: 11/24/2022] Open
Abstract
Introduction Desmoid tumors are benign fibrous entities developed from connective tissue, and they result from muscle fascia or aponeurosis. Surgical excision is the main pillar of treatment. Case report A 29-year-old female patient presented with a left postero-lateral cervical swelling that had been evolving for 11 months. Cervical MRI showed a mass at the expense of the trapezium muscle measuring 41 × 68 × 81 mm. A biopsy of the mass was performed, concluding a desmoid tumor. The patient underwent a large resection of the tumor involving the left trapezius muscle. An immunohistochemistry staining was performed, demonstrating cytoplasmic labeling with anti AML antibody, and nuclear labeling of tumor cells with anti-beta-catenin antibody. The case was analyzed by a multidisciplinary committee, and it was decided to follow the patient for surveillance. Discussion The localization of desmoid tumors in the trapezius muscle is extremely rare. The main risk of these benign lesions is infiltration of surrounding tissue leading to morbidity and mortality. Recurrence is a main feature of these tumors, even if complete excision has been performed. Unresectable desmoid tumors require medical and non-surgical treatment such as hormone therapy (tamoxifen), and chemotherapy with vinblastine and methotrexate. Conclusion Desmoid tumor of trapezius muscle is classified as benign without metastatic power, and the main risk is infiltration of surrounding tissue.
Desmoid tumors are benign fibrous entities developed from connective tissue. Desmoid tumors comprise only 12–15% of cases in the head and neck, and can occur at any age. The localization of desmoid tumors in the trapezius muscle is extremely rare. The symptomatology of a desmoid trapezius muscle tumor remains aspecific. Complete resection with clear miscroscopic margins is the treatment of choice for resectable symptomatic desmoid tumors.
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Kuwabara H, Katayanagi S, Koganezawa I, Nakagawa M, Katsumata K, Tsuchida A, Kawachi S. Sporadic intra-abdominal desmoid tumor with a very unusual onset: two case reports. J Med Case Rep 2021; 15:457. [PMID: 34526110 PMCID: PMC8444561 DOI: 10.1186/s13256-021-03058-z] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/05/2021] [Accepted: 08/11/2021] [Indexed: 01/10/2023] Open
Abstract
Background Intra-abdominal desmoid tumors are rare soft tissue tumors that arise mainly in the mesentery and pelvis. Their etiology may include genetic mutations, estrogen-associated changes after childbirth, and mechanical factors such as a history of abdominal surgery. However, there are cases of intra-abdominal desmoid tumors that develop in the absence of such causes. Since they are rare, diagnosis is often difficult based on clinical findings. We encountered two cases of patients with sporadic intra-abdominal desmoid tumors with a very unusual onset and contrasting features. Case presentation The first patient was a 51-year-old asian man who presented with sudden onset of abdominal pain. He was referred to our department because of a giant tumor detected on abdominal ultrasonography. Imaging revealed a 19-cm tumor with internal tumoral hemorrhage; however, no definitive diagnosis was made. Tumor resection was performed for diagnostic and therapeutic purposes. The second patient was a 41-year-old asian man, and right hydronephrosis was detected on abdominal ultrasonography during a periodic medical checkup. We diagnosed invasion of the primary mesenteric tumor into the right ureter using diagnostic imaging and performed ileocecal resection with partial right ureteral resection for a definitive diagnosis and therapeutic purposes. Although the tumors of both patients had developed from the ileal mesentery, the tumors were substantially different from each other based on their imaging findings, macroscopic morphology, and progression pattern. Meanwhile, they showed similar pathological characteristics. Both consisted of bundles of collagen fibrils of spindle-shaped fibroblasts with low cell atypia. Moreover, they were diagnosed as desmoid tumors using positive immunohistochemical staining for β-catenin. Conclusions Neither patient had susceptibility factors for desmoid tumors, and to our knowledge, there have been very few reports to date of intra-abdominal desmoid tumors that were diagnosed because of acute abdominal pain caused by tumoral hemorrhage or asymptomatic obstructive uropathy. Furthermore, it is clinically interesting that the two patients showed contrasting progression patterns and imaging findings. Intra-abdominal desmoid tumors are rare and may present with various symptoms and findings similar to those observed in our patients. Diagnosis therefore requires experience and knowledge that is not bound by preconceptions.
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Affiliation(s)
- Hiroshi Kuwabara
- Department of Digestive and Transplantation Surgery, Tokyo Medical University Hachioji Medical Center, 1163 Tatemachi, Hachioji, Tokyo, 193-0998, Japan. .,Department of Gastrointestinal and Pediatric Surgery, Tokyo Medical University, 6-7-1 Nishishinjuku, Shinjuku Ward, Tokyo, 160-0012, Japan.
| | - Sou Katayanagi
- Department of Digestive and Transplantation Surgery, Tokyo Medical University Hachioji Medical Center, 1163 Tatemachi, Hachioji, Tokyo, 193-0998, Japan
| | - Itsuki Koganezawa
- Department of Digestive and Transplantation Surgery, Tokyo Medical University Hachioji Medical Center, 1163 Tatemachi, Hachioji, Tokyo, 193-0998, Japan
| | - Masashi Nakagawa
- Department of Digestive and Transplantation Surgery, Tokyo Medical University Hachioji Medical Center, 1163 Tatemachi, Hachioji, Tokyo, 193-0998, Japan
| | - Kenji Katsumata
- Department of Gastrointestinal and Pediatric Surgery, Tokyo Medical University, 6-7-1 Nishishinjuku, Shinjuku Ward, Tokyo, 160-0012, Japan
| | - Akihiko Tsuchida
- Department of Gastrointestinal and Pediatric Surgery, Tokyo Medical University, 6-7-1 Nishishinjuku, Shinjuku Ward, Tokyo, 160-0012, Japan
| | - Shigeyuki Kawachi
- Department of Digestive and Transplantation Surgery, Tokyo Medical University Hachioji Medical Center, 1163 Tatemachi, Hachioji, Tokyo, 193-0998, Japan
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Sommer F. Rare Diseases of the Nose, the Paranasal Sinuses, and the Anterior Skull Base. Laryngorhinootologie 2021; 100:S1-S44. [PMID: 34352902 PMCID: PMC8354577 DOI: 10.1055/a-1331-2469] [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] [Indexed: 11/13/2022]
Abstract
Due to their low incidence and thus resulting limited diagnostic criteria as well as therapeutic options, rare diseases of the nose, the paranasal sinuses, and the anterior skull base are a significant challenge. The value as of which a disease has to be considered as rare amounts to a maximum of 5 patients per 10 000 people. Within these diseases, however, there are extreme differences. Some rare or orphan diseases like for example the inverted papilloma belong to regularly diagnosed and treated diseases of larger departments of oto-rhino-laryngology whereas other rare diseases and malformations have only been described in less than 100 case reports worldwide. This fact emphasizes the necessity of bundling the available experience of diagnostics and therapy. The present article gives an overview about rare diseases of the nose, the paranasal sinuses, and the anterior skull base from the field of diseases/syndromes of the olfactory system, malformations of the nose and paranasal sinuses, ventilation and functional disorders as well as benign and malignant tumors. The classification and data on diagnostic and therapeutic options were established based on the current literature.
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Affiliation(s)
- Fabian Sommer
- Klinik für Hals-Nasen-Ohrenheilkunde, Kopf- und Hals-Chirurgie, Universitätsklinik Ulm
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Zhang Z, Shi J, Yang T, Liu T, Zhang K. Management of aggressive fibromatosis. Oncol Lett 2021; 21:43. [PMID: 33262835 PMCID: PMC7693298 DOI: 10.3892/ol.2020.12304] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/30/2020] [Accepted: 10/30/2020] [Indexed: 01/12/2023] Open
Abstract
Aggressive fibromatosis or desmoid tumor is a rare disease resulting from fibroblasts which do not metastasize. However, desmoid tumors belong to low-grade malignant tumors since they have high potential to infiltrate surrounding tissues, causing high local recurrence rates and may affect surrounding organs, threatening life quality and expectancy. Although surgery, watch and wait, radiotherapy, chemotherapy, high intensity focused ultrasound, ablation techniques or several agents have all been frequently investigated for the treatment of this type of disease, none are deemed as standard therapy for high recurrence rates that have been supported by any data. The present review retrieved literature on treatment options for desmoids to summarize the latest treatment modalities and refine their efficacy, as well as their side effects, in order to provide a more comprehensive treatment reference for clinicians.
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Affiliation(s)
- Zhijun Zhang
- Department of Colorectal and Anal Surgery, The Second Hospital of Jilin University, Changchun, Jilin 130012, P.R. China
| | - Jian Shi
- Department of Colorectal and Anal Surgery, The Second Hospital of Jilin University, Changchun, Jilin 130012, P.R. China
| | - Tao Yang
- Department of Colorectal and Anal Surgery, The Second Hospital of Jilin University, Changchun, Jilin 130012, P.R. China
| | - Tongjun Liu
- Department of Colorectal and Anal Surgery, The Second Hospital of Jilin University, Changchun, Jilin 130012, P.R. China
| | - Kai Zhang
- Department of Colorectal and Anal Surgery, The Second Hospital of Jilin University, Changchun, Jilin 130012, P.R. China
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Mesenteric infiltrative desmoid tumor bordering the pancreas and mimicking liposarcoma. Clin Res Hepatol Gastroenterol 2020; 44:625-627. [PMID: 32014388 DOI: 10.1016/j.clinre.2020.01.005] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/02/2020] [Accepted: 01/08/2020] [Indexed: 02/04/2023]
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Local Control and Analgesic Efficacy of Percutaneous Cryoablation for Desmoid Tumors. Cardiovasc Intervent Radiol 2019; 43:110-119. [PMID: 31471720 DOI: 10.1007/s00270-019-02323-5] [Citation(s) in RCA: 23] [Impact Index Per Article: 3.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/04/2019] [Accepted: 08/22/2019] [Indexed: 12/22/2022]
Abstract
OBJECTIVE Cryoablation is being more and more often used to treat desmoid tumors. We report our experience of cryoablation as a local and analgesic treatment for inoperable or recurring desmoid tumors. METHODS This study includes 34 patients who underwent percutaneous cryoablation of 41 desmoid tumors between July 2012 and September 2016. All patients underwent pain assessment using a visual analog scale (VAS) and preoperative imaging. All cryoablation procedures were performed using the same cryoablation system. Patients received clinical and radiological follow-up at 6 months post-procedure, with pain-rating and adverse events being recorded. A long-term follow-up was achieved until 31 December 2018. Disease-free survival at 3 years was also recorded. Radiological tumor response was determined by tumor measurements using RECIST 1.1. RESULTS Twelve patients benefitted from curative treatment on 100% of the tumor volume, but 22 patients received debulking treatment because of the risk of neighboring structures. Two patients had a postoperative hematoma grade 2 of the CIRSE classification system for complications, and two patients had grade 4 complications involving palsy of the common fibular nerve. Disease-free survival at 3 years was 42.2%. The mean VAS pain scores were 5.7 and 2.4 at pretreatment and 6 months, respectively, showing a mean reduction of 3.3 (p < 0.001). At 6 months, all measured tumor dimensions were significantly lower than pretreatment. CONCLUSION Cryoablation is an effective therapeutic option for the local treatment and for the analgesic management of desmoid tumors.
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Muneer M, Badran S, Zahid R, Abdelmageed A, AlDulaimi MM. Recurrent Desmoid Tumor with Intra-Abdominal Extension After Abdominoplasty: A Rare Presentation. AMERICAN JOURNAL OF CASE REPORTS 2019; 20:953-956. [PMID: 31270310 PMCID: PMC6621933 DOI: 10.12659/ajcr.916227] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
BACKGROUND Desmoid tumors are fibrous neoplasms that originate from the musculoaponeurotic structures in the body. Abdominal wall desmoid tumors are rare, but they can be locally aggressive, with high incidence of recurrence. These tumors are more common in young, fertile women. They frequently occur during or after pregnancy. CASE REPORT We present the case of a 63-year-old post-menopausal woman with a desmoid tumor of the anterior abdominal wall. She had no relevant family history. During abdominoplasty, an incidental mass was excised and biopsied, and was identified as a desmoid tumor with free margins. One year later, the patient presented with vague abdominal discomfort and feeling of heaviness. An incision was made through the previous abdominoplasty scar to maintain the aesthetic outcome. A large mass, arising from the abdominal wall and extending intra-abdominally, was excised and was determined to be a recurrent desmoid tumor. CONCLUSIONS Recurrent anterior abdominal wall desmoid tumors in post-menopausal women are rare and locally aggressive, with a high risk of recurrence. During abdominal wall repair in abdominoplasty, desmoid tumor filaments might seed deep intra-abdominally. Therefore, it is necessary to take adequate safe margins before abdominal wall repair. Post-operatively, surgeons should keep a high index of suspicion for tumor recurrence.
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Affiliation(s)
- Mohammed Muneer
- Department of Plastic Surgery, Hamad Medical Corporation, Doha, Qatar.,Department of Surgery, Weill Cornel Medical College, Doha, Qatar
| | - Saif Badran
- Department of Plastic Surgery, Hamad Medical Corporation, Doha, Qatar
| | - Rehan Zahid
- Department of Plastic Surgery, Hamad Medical Corporation, Doha, Qatar
| | - Amal Abdelmageed
- Department of Health and Life Sciences, Hamad bin Khalifa University, Doha, Qatar
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Gong LH, Liu WF, Ding Y, Geng YH, Sun XQ, Huang XY. Diagnosis and Differential Diagnosis of Desmoplastic Fibroblastoma by Clinical, Radiological, and Histopathological Analyses. Chin Med J (Engl) 2019; 131:32-36. [PMID: 29271377 PMCID: PMC5754955 DOI: 10.4103/0366-6999.221274] [Citation(s) in RCA: 18] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/20/2022] Open
Abstract
Background: Desmoplastic fibroblastoma (collagenous fibroma) is an uncommon benign soft-tissue tumor, rarely involving bone. It shares some overlapping features with other infiltrate tumors, such as desmoid-type fibromatosis, neurofibroma, and low-grade fibromyxoid sarcoma. The misdiagnosis may cause unnecessary surgical overtreatment, especially for those involving bone. In order to deepen the understanding of the diagnosis and differential diagnosis of desmoplastic fibroblastoma, we planned to analyze the clinical, radiological, and histopathological features and the outcome of desmoplastic fibroblastoma on the basis of case analysis and literature review. Methods: Sixteen cases were retrieved from the surgical pathology records from May 2011 to April 2016 in the Department of Pathology in Beijing Jishuitan Hospital. Formalin-fixed, paraffin-embedded specimens of 16 cases of desmoplastic fibroblastoma were collected. Hematoxylin and eosin stain and immunohistochemistry were used to observe the histological features of desmoplastic fibroblastoma of soft tissue and bone. The images for diagnosis obtained from the ultrasonic examination, X-ray, magnetic resonance imaging, and computed tomography were used to observe the radiological features. Related literatures were retrieved from the PubMed and CNKI databases. Results: Sixteen cases of desmoplastic fibroblastoma of soft tissue were located in the hand (n = 7), foot (n = 4), upper arm (n = 1), shoulder (n = 1), forearm (n = 2), and one case occurred in the proximal femur. Age ranged from 32 to 82 years (median age: 58 years). There were six females and ten males. Histologically, the lesions of soft tissue appeared as well-circumscribed masses with abundant collagenous matrix and low vascularity. Tumor cells were stellate- or spindle-shaped and uniformly distributed within the extracellular matrix. In five cases, the desmoplastic fibroblastoma were found to have infiltrated into the skeletal muscle tissue. In one case of desmoplastic fibroblastoma of bone, radiographs revealed osteolytically well-defined lesion. Immunohistochemistry stain showed that vimentin and smooth muscle actin were positive in all cases of desmoplastic fibroblastoma. Conclusions: Desmoplastic fibroblastoma (collagenous fibroma) has prominent clinical, histopathological, and radiological features. Before the differential diagnosis from other tumors is obtained by thorough analysis and comparison of the similar and different characteristics, the appropriate surgical management and accurate prognosis evaluation could not be delivered to the patient.
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Affiliation(s)
- Li-Hua Gong
- Department of Pathology, Beijing Jishuitan Hospital, The Fourth Medical College of Peking University, Beijing 100035, China
| | - Wei-Feng Liu
- Department of Orthopedic Oncology, Beijing Jishuitan Hospital, The Fourth Medical College of Peking University, Beijing 100035, China
| | - Yi Ding
- Department of Pathology, Beijing Jishuitan Hospital, The Fourth Medical College of Peking University, Beijing 100035, China
| | - Yue-Hang Geng
- Department of Pathology, Peking University Health Science Center, Beijing 100191, China
| | - Xiao-Qi Sun
- Department of Pathology, Beijing Jishuitan Hospital, The Fourth Medical College of Peking University, Beijing 100035, China
| | - Xiao-Yuan Huang
- Department of Pathology, Beijing Jishuitan Hospital, The Fourth Medical College of Peking University, Beijing 100035, China
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Dioguardi Burgio M, Ronot M, Vilgrain V. Rare Solid Tumor of the Exocrine Pancreas: A Pictorial Review. Semin Ultrasound CT MR 2019; 40:483-499. [PMID: 31806147 DOI: 10.1053/j.sult.2019.04.007] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
Numerous other solid primary neoplasms may arise from the pancreas besides primary ductal adenocarcinomas and neuroendocrine tumors. Although diagnosis can be difficult because of the very low incidence of these tumors, knowledge of several, typical, epidemiologic, biological, and imaging features can help obtain a correct diagnosis. This pictorial review describes the features of solid rare primary pancreatic neoplasms on computed tomography and magnetic resonance imaging focusing on characteristics that can help radiologists differentiate them from classical forms of ductal pancreatic adenocarcinoma and neuroendocrine tumors. Cystic pancreatic neoplasms are beyond the scope of the current review.
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Affiliation(s)
- Marco Dioguardi Burgio
- Department of Radiology, University Hospitals Paris Nord Val de Seine, Beaujon, Clichy, Hauts-de-Seine, France; INSERM U1149, Centre de Recherche Biomédicale Bichat-Beaujon, CRB3, Paris, France.
| | - Maxime Ronot
- Department of Radiology, University Hospitals Paris Nord Val de Seine, Beaujon, Clichy, Hauts-de-Seine, France; INSERM U1149, Centre de Recherche Biomédicale Bichat-Beaujon, CRB3, Paris, France; University Paris Diderot, Sorbonne Paris Cité, Paris, France
| | - Valérie Vilgrain
- Department of Radiology, University Hospitals Paris Nord Val de Seine, Beaujon, Clichy, Hauts-de-Seine, France; INSERM U1149, Centre de Recherche Biomédicale Bichat-Beaujon, CRB3, Paris, France; University Paris Diderot, Sorbonne Paris Cité, Paris, France
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Abstract
INTRODUCTION Desmoid tumours of the head and neck, also known as fibromatosis, are rare, locally invasive benign tumours with high recurrence rate, causing considerable morbidity. Complete surgical excision of desmoid tumours is considered to be the only effective treatment. CASE REPORT We present a case of fibromatosis of the right posterolateral region of the neck in a 56-year-old woman who presented with right neck mass. The patient underwent complete excision of the tumour with no adjuvant therapy. No recurrence or neurological deficit was observed 2 years after surgery. CONCLUSION Although desmoid tumour is a benign neoplasm with no metastatic potential, treatment is challenging due to its aggressive, infiltrative behaviour with a tendency to recur.
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Abstract
Desmoid tumors (fibromatosis) are rare benign tumors, they arise from musculoaponeurotic structures throughout the body. They are locally infiltrative, resulting in a high rate of local recurrence following surgical resection. Due to the rarity of these tumors in the head and neck region, we report a case of a patient with a desmoid tumor in the upper neck that was diagnosed and treated in our institution, to increase the awareness of the Otolaryngology-Head and Neck surgeons, and report available treatment options of this condition.
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Affiliation(s)
- Ameen Z Alherabi
- Department of Otolaryngology-Head & Neck Surgery, Umm Al-Qura University, Makkah, Kingdom of Saudi Arabia. E-mail.
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Long-term outcomes of 47 patients with aggressive fibromatosis of the chest treated with surgery. Eur J Surg Oncol 2016; 42:1693-1698. [PMID: 27425579 DOI: 10.1016/j.ejso.2016.06.396] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/23/2015] [Revised: 05/30/2016] [Accepted: 06/16/2016] [Indexed: 01/16/2023] Open
Abstract
AIMS The purpose of the study was to review a large series of chest aggressive fibromatosis (AF) cases with an emphasis on the outcomes of different degrees of resection and the value of postoperative radiotherapy. MATERIALS AND METHODS The records of patients with chest AF treated at our hospital from 1982 to 2014 were retrospectively reviewed. Recurrence rates and non-disease survival (NDS) times were compared between the R0, R1, and R2 resection groups. RESULTS Forty-seven cases of chest AF were treated during the study period (21 men, 26 women), with an average age at diagnosis of 40 years (range, 9-77 years). One patient died before surgery, and 46 patients received a total of 85 resections. Forty-one patients had complete follow-up data, and the average follow-up time was 125.6 months (range, 11-524 months). Recurrence rates were 6.7%, 92.9%, and 100% for the R0, R1, and R2 resection groups, respectively, and the R0 recurrence rate was significantly lower than the R1 and R2 rates (both P values < 0.001). The NDS time of the R0, R1, and R2 groups was 80.3 ± 64.8, 23.6 ± 38.7, and 9.8 ± 10.8 months, respectively; the NDS time of the R0 group was significantly longer than that of the R1 and R2 groups (both P values < 0.01). Within each resection type, no significant differences were found in the recurrence rates of patients having surgery alone compared with those receiving surgery and radiotherapy (all P values > 0.05). CONCLUSION R0 resection is the most effective treatment for chest AF. Postoperative radiotherapy did not reduce the recurrence rate.
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Extra-abdominal desmoid fibromatosis: A review of management, current guidance and unanswered questions. Eur J Surg Oncol 2016; 42:1071-83. [PMID: 26965303 DOI: 10.1016/j.ejso.2016.02.012] [Citation(s) in RCA: 49] [Impact Index Per Article: 5.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/24/2015] [Revised: 01/30/2016] [Accepted: 02/11/2016] [Indexed: 12/17/2022] Open
Abstract
Extra abdominal desmoid fibromatosis is a complex condition with many recognised treatments including active observation, hormonal therapy, chemotherapy, radiotherapy and surgical resection. There is large variation in the natural history of individual desmoid tumours, with some cases progressing aggressively and others regressing spontaneously when observed alone. This combined with an absence of accurate clinical predictors of a desmoid tumour's behaviour has led to difficulties in identifying which patients would benefit most from aggressive treatment, and which could be adequately managed with a policy of active observation alone. This review explores the aetiology and common presentation of extra-abdominal desmoid fibromatosis including the condition's histopathological, clinical and radiological characteristics. The current evidence for potential predictors of desmoid tumour behaviour is also reviewed, along with the indications and evidence for the multitude of treatments available. We also summarise the published guidelines that are currently available for oncologists and surgeons managing extra-abdominal desmoid fibromatosis, and highlight some of the unanswered questions that need to be addressed to optimise the management of this condition.
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Kamali F, Wang WL, Guadagnolo BA, Fox PS, Lewis VO, Lazar AJ, Conley AP, Ravi V, Toliyat M, Ladha HS, Hobbs BP, Amini B. MRI may be used as a prognostic indicator in patients with extra-abdominal desmoid tumours. Br J Radiol 2015; 89:20150308. [PMID: 26577289 DOI: 10.1259/bjr.20150308] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/05/2022] Open
Abstract
OBJECTIVE To determine the association of MRI features of extra-abdominal desmoid tumours (DTs) with prognosis. METHODS MRIs for 90 patients with DT were retrospectively reviewed for imaging features associated with biological behaviour. The primary end point was progression (for lesions managed with chemotherapy, radiation therapy and observation) or recurrence (following surgery). Time to event was studied using univariate and multivariable Cox proportional hazards regression models when accounting for demographic, clinicopathological and imaging variables. Kaplan-Meier plots were used to estimate event-free rate (EFR). RESULTS Univariate analysis revealed a significant relationship between EFR and treatment, location and compartment of origin [subcutaneous (SC), superficial fascial, intramuscular (IM) and deep fascial/intermuscular]. None of the imaging features commonly associated with biological behaviour of DTs (e.g., shape, enhancement, T2 signal etc.) or surgical margins (in surgical cases) was associated with EFR. Multivariate analysis showed that treatment modality and compartment of origin were independent predictors of EFR. Superficial and deep fascial lesions had a significantly worse EFR as a group [hazard ratio: 3.9; 95% confidence interval (CI): 1.83-8.32; p = 0.0004] than did the SC and IM lesions as a group. 5-year EFR for the fascial lesions was 18% (95% CI: 6-36%), compared with 57% (95% CI: 25-79%) for the SC and IM groups. CONCLUSION Intramuscular or SC DTs may be associated with improved prognosis. If validated on multireader and prospective studies, these results can provide for rapid risk stratification at the time of initial MRI. ADVANCES IN KNOWLEDGE This work has shown that imaging features commonly associated with biological activity of desmoid tumours (e.g. shape, T2 signal and enhancement) do not appear to be associated with prognosis in patients undergoing a variety of treatment modalities. The compartment of origin of the lesion, which can be determined on pre-operative MRI, was shown to be associated with prognosis and can allow for risk stratification in patients with DTs.
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Affiliation(s)
- Firouzeh Kamali
- 1 Department of Diagnostic Radiology, University of Texas M.D. Anderson Cancer Center, Houston, TX, USA.,2 Department of Diagnostic Radiology, University of Texas at Houston, Houston, TX, USA
| | - Wei-Lien Wang
- 3 Department of Pathology, University of Texas M.D. Anderson Cancer Center, Houston, TX, USA
| | - B A Guadagnolo
- 4 Department of Radiation Oncology, University of Texas M.D. Anderson Cancer Center, Houston, TX, USA.,5 Departments of Health Services Research, University of Texas M.D. Anderson Cancer Center, Houston, TX, USA
| | - Patricia S Fox
- 6 Departments of Biostatistics, University of Texas M.D. Anderson Cancer Center, Houston, TX, USA
| | - Valerae O Lewis
- 7 Departments of Orthopedic Oncology, University of Texas M.D. Anderson Cancer Center, Houston, TX, USA
| | - Alexander J Lazar
- 3 Department of Pathology, University of Texas M.D. Anderson Cancer Center, Houston, TX, USA
| | - Anthony P Conley
- 8 Departments of Medical Oncology, University of Texas M.D. Anderson Cancer Center, Houston, TX, USA
| | - Vinod Ravi
- 8 Departments of Medical Oncology, University of Texas M.D. Anderson Cancer Center, Houston, TX, USA
| | - Mohammad Toliyat
- 9 Department of Diagnostic Radiology, University of Texas Southwestern Medical Center, Dallas, TX, USA
| | - Harshad S Ladha
- 1 Department of Diagnostic Radiology, University of Texas M.D. Anderson Cancer Center, Houston, TX, USA
| | - Brian P Hobbs
- 5 Departments of Health Services Research, University of Texas M.D. Anderson Cancer Center, Houston, TX, USA
| | - Behrang Amini
- 1 Department of Diagnostic Radiology, University of Texas M.D. Anderson Cancer Center, Houston, TX, USA
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17
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Jia C, Tian B, Dai C, Wang X, Bu X, Xu F. Idiopathic desmoid-type fibromatosis of the pancreatic head: case report and literature review. World J Surg Oncol 2014; 12:103. [PMID: 24755337 PMCID: PMC4032157 DOI: 10.1186/1477-7819-12-103] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/26/2013] [Accepted: 04/07/2014] [Indexed: 12/17/2022] Open
Abstract
Desmoid-type fibromatosis (DTF) is an uncommon nonmetastatic fibrous neoplasm. Sporadic intraperitoneal DTF is rarely described in current literature. We herein report a case of DTF of unknown cause involving the pancreatic head. A 41-year-old man presented with recurrent epigastric pain and weight loss. An abdominal computed tomography scan showed a well-delineated solid cystic mass inside the pancreatic head. Pylorus-preserving pancreaticoduodenectomy was performed due to the patient's debilitating symptoms and suspected malignancy. The pathological examination revealed massive fibroblastic proliferation arising from the musculoaponeurotic tissues, consistent with a diagnosis of DTF. Immunohistochemical phenotyping determined positive immunoreactivity to vimentin and β-catenin, but negative immunoreactivity to smooth muscle actin, CD117, CD34, or S-100, confirming the diagnosis of DTF. No local recurrence or distant metastasis was found during a 24-month follow-up. Radical resection is recommended as first-line treatment for pancreatic DTF. Long-term follow-up studies are required to establish the prognosis of pancreatic DTF.
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Affiliation(s)
- Changjun Jia
- Department of General Surgery, Shengjing Hospital, China Medical University, 36 Sanhao Street, Shenyang 110004, China
| | - Baoling Tian
- Department of Pathology, Shengjing Hospital, China Medical University, 36 Sanhao Street, Shenyang 110004, China
| | - Chaoliu Dai
- Department of General Surgery, Shengjing Hospital, China Medical University, 36 Sanhao Street, Shenyang 110004, China
| | - Xinlu Wang
- Department of Ultrasound Medicine, Shengjing Hospital, China Medical University, 36 Sanhao Street, Shenyang 110004, China
| | - Xianmin Bu
- Department of General Surgery, Shengjing Hospital, China Medical University, 36 Sanhao Street, Shenyang 110004, China
| | - Feng Xu
- Department of General Surgery, Shengjing Hospital, China Medical University, 36 Sanhao Street, Shenyang 110004, China
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18
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Hamada S, Urakawa H, Kozawa E, Futamura N, Ikuta K, Shimoyama Y, Nakamura S, Ishiguro N, Nishida Y. Nuclear expression of β-catenin predicts the efficacy of meloxicam treatment for patients with sporadic desmoid tumors. Tumour Biol 2014; 35:4561-6. [PMID: 24390670 DOI: 10.1007/s13277-013-1600-7] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/22/2013] [Accepted: 12/23/2013] [Indexed: 11/25/2022] Open
Abstract
This study aimed to determine the prevalence of β-catenin nuclear positivity as a prognostic factor in patients with desmoid tumors (DTs) treated with meloxicam, a cyclooxygenase-2 (COX-2) selective inhibitor. Between 2003 and 2012, consecutive 31 patients with extraabdominal, sporadic DTs were prospectively treated with meloxicam as a systemic medical therapy. Immunohistochemistry was performed on formalin-fixed material to quantify the nuclear expression of β-catenin and Ki-67, and cytoplasmic expression of COX-2. All clinicopathological characteristics including the intensity of immunohistochemical staining were analyzed with respect to their prognostic value for meloxicam treatment. Of the 31 patients with meloxicam treatment, there was 1 with complete remission (CR), 7 with partial remission (PR), 12 with stable disease (SD), and 11 with progressive disease (PD). Higher nuclear expression of β-catenin was significantly associated with a poor response (PD/SD) (p = 0.017). The positivity of COX-2 and Ki-67 and none of the other clinical variables were associated with prognosis. The nuclear expression of β-catenin can predict the efficacy of meloxicam treatment for patients with sporadic DTs.
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Affiliation(s)
- Shunsuke Hamada
- Department of Orthopedic Surgery, Nagoya University Graduate School and School of Medicine, 65 Tsurumai, Showa, Nagoya, Aichi, 466-8550, Japan
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19
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Wood TJ, Quinn KM, Farrokhyar F, Deheshi B, Corbett T, Ghert MA. Local control of extra-abdominal desmoid tumors: systematic review and meta-analysis. Rare Tumors 2013; 5:e2. [PMID: 23772301 PMCID: PMC3682454 DOI: 10.4081/rt.2013.e2] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/10/2012] [Revised: 08/08/2012] [Accepted: 09/04/2012] [Indexed: 12/17/2022] Open
Abstract
The local control of desmoid tumors constitutes a continuing treatment dilemma due to its high recurrence rates. The purpose of this systematic review was to critically examine the current treatment of these rare tumors and to specifically evaluate the local failure and response rates of surgery, radiation and systemic therapy. We comprehensively searched the literature for relevant studies across Cinahl, Embase, Medline and the Cochrane databases. Articles were categorized as surgery, radiation, surgery + radiation and systemic therapy (including cytotoxic and non cytotoxic). Methodological quality of included studies was assessed using the Newcastle-Ottawa Scale. Pooled odd ratios (OR) for comparative studies and weighted proportions with 95% confidence intervals (CI) are reported. Thirty-five articles were included in the final analysis. Weighted mean local failure rates were 22% [95% CI (16–28%)], 35% [95% CI (26–44%)] and 28% [95% CI (18–39%)] for radiation alone, surgery alone and surgery + radiation respectively. In the analysis of comparative studies, surgery and radiation in combination had lower local failure rates than radiation alone [OR 0.7 (0.4, 1.2)] and surgery alone [OR 0.7 (0.4, 1.0)]. Weighted mean stable disease rates were 91% [95% CI (85–96%)] and 52% [95% CI (38–65%)] for non cytotoxic and cytotoxic chemotherapy respectively. The current evidence suggests that surgery alone has a consistently high rate of local recurrence in managing extra-abdominal desmoid tumors. Radiation therapy in combination with surgery improves local control rates. However, the limited data on systemic therapy for this rare tumor suggests the benefit of using both cytotoxic and non cytotoxic chemotherapy to achieve stable disease.
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Affiliation(s)
- Thomas J Wood
- Michael G. DeGroote School of Medicine, ; Department of Surgery
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20
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Siqueira MG, Tavares PL, Martins RS, Heise CO, Foroni LHL, Bordalo M, Falzoni R. Management of desmoid-type fibromatosis involving peripheral nerves. ARQUIVOS DE NEURO-PSIQUIATRIA 2013; 70:514-9. [PMID: 22836457 DOI: 10.1590/s0004-282x2012000700008] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Received: 12/17/2011] [Accepted: 12/30/2011] [Indexed: 11/22/2022]
Abstract
Desmoid-type fibromatosis is an uncommon and aggressive neoplasia, associated with a high rate of recurrence. It is characterized by an infiltrative but benign fibroblastic proliferation occurring within the deep soft tissues. There is no consensus about the treatment of those tumors. We present a surgical series of four cases, involving the brachial plexus (two cases), the median nerve and the medial brachial cutaneous nerve. Except for the last case, they were submitted to multiple surgical procedures and showed repeated recurrences. The diagnosis, the different ways of treatment and the prognosis of these tumoral lesions are discussed. Our results support the indication of radical surgery followed by radiotherapy as probably one of the best ways to treat those controversial lesions.
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Affiliation(s)
- Mario G Siqueira
- Peripheral Nerve Surgery Unit, Department of Neurosurgery, Medical School, University of São Paulo, Rua Maestro Cardim 592, São Paulo, SP, Brazil.
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21
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Seki M, Koga R, Saiura A, Nakagawa K, Kawabata K, Kanda H, Machinami M, Nakagawa M. Mesenteric fibromatosis of the transverse colon with the reconstruction of the superior mesenteric arteries: report of a case. Surg Today 2012; 42:703-7. [PMID: 22592908 DOI: 10.1007/s00595-012-0187-8] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/16/2011] [Accepted: 08/15/2011] [Indexed: 01/02/2023]
Abstract
In general, with large mesenteric tumors it may be rather difficult to determine whether infiltration into adjacent large vessels occurred. We wish to stress the importance of preparation for microsurgery when a huge lesion appears close to a large artery in preoperative images, based on our experience of successful microscopical reconstruction of a superior mesenteric artery (SMA) and marked improvement of blocked vascular flow to the small intestine during the surgery. We have experienced a case of mesenteric fibromatosis (MF) invading the SMA and vein, contrary to preoperative expectation. The patient underwent extirpation of a MF, 21 cm in size, with reconstruction of the SMA by microsurgery. The sacrificed small intestine was only 80 cm of the distal ileum with the benefit of microscopic anastomosis between the SMA and a major jejunal artery. Preparations for microscopic surgery must be made with resection of large lesions, because involvement of mesenteric large vessels may be expected. It is possible for microsurgery to extend indications for surgical resection of huge mesenteric tumors.
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Affiliation(s)
- Makoto Seki
- Department of Surgery, Cancer Institute of the Japanese Foundation for Cancer Research, 3-10-6 Ariake, Koto-ku, Tokyo, 135-8550, Japan.
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22
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Salas S, Dufresne A, Bui B, Blay JY, Terrier P, Ranchere-Vince D, Bonvalot S, Stoeckle E, Guillou L, Le Cesne A, Oberlin O, Brouste V, Coindre JM. Prognostic Factors Influencing Progression-Free Survival Determined From a Series of Sporadic Desmoid Tumors: A Wait-and-See Policy According to Tumor Presentation. J Clin Oncol 2011; 29:3553-8. [DOI: 10.1200/jco.2010.33.5489] [Citation(s) in RCA: 251] [Impact Index Per Article: 17.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022] Open
Abstract
Purpose Desmoid tumors are mesenchymal fibroblastic/myofibroblastic proliferations with locoregional aggressiveness and high ability to recur after initial treatment. We present the results of the largest series of sporadic desmoid tumors ever published to determine the prognostic factors of these rare tumors. Patients and Methods Four hundred twenty-six patients with a desmoid tumor at diagnosis were included, and the following parameters were studied: age, sex, delay between first symptoms and diagnosis, tumor size, tumor site, previous history of surgery or trauma in the area of the primary tumor, surgical margins, and context of abdominal wall desmoids in women of child-bearing age during or shortly after pregnancy. We performed univariate and multivariate analysis for progression-free survival (PFS). Results In univariate analysis, age, tumor size, tumor site, and surgical margins (R2 v R0/R1) had a significant impact on PFS. PFS curves were not significantly different for microscopic assessment of surgical resection quality (R0 v R1). In multivariate analysis, age, tumor size, and tumor site had independent values. Three prognostic groups for PFS were defined on the basis of the number of independent unfavorable prognostic factors (0 or 1, 2, and 3). Conclusion This study clearly demonstrates that there are different prognostic subgroups of desmoid tumors that could benefit from different therapeutic strategies, including a wait-and-see policy.
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Affiliation(s)
- Sébastien Salas
- Sébastien Salas, L'Institut National de la Santé et de la Recherche Médicale (INSERM) U916, Bergonié Institute, Bordeaux; Binh Bui, Eberhard Stoeckle, Véronique Brouste, and Jean Michel Coindre, Bergonié Institute; Jean-Michel Coindre, University Victor Ségalen, Bordeaux; Sébastien Salas, Timone Hospital, Marseille; Armelle Dufresne, Jean-Yves Blay, and Dominique Ranchere-Vince, Leon Berard Center, Lyon; Philippe Terrier, Sylvie Bonvalot, Axel Le Cesne and Odile Oberlin, Gustave Roussy Institute,
| | - Armelle Dufresne
- Sébastien Salas, L'Institut National de la Santé et de la Recherche Médicale (INSERM) U916, Bergonié Institute, Bordeaux; Binh Bui, Eberhard Stoeckle, Véronique Brouste, and Jean Michel Coindre, Bergonié Institute; Jean-Michel Coindre, University Victor Ségalen, Bordeaux; Sébastien Salas, Timone Hospital, Marseille; Armelle Dufresne, Jean-Yves Blay, and Dominique Ranchere-Vince, Leon Berard Center, Lyon; Philippe Terrier, Sylvie Bonvalot, Axel Le Cesne and Odile Oberlin, Gustave Roussy Institute,
| | - Binh Bui
- Sébastien Salas, L'Institut National de la Santé et de la Recherche Médicale (INSERM) U916, Bergonié Institute, Bordeaux; Binh Bui, Eberhard Stoeckle, Véronique Brouste, and Jean Michel Coindre, Bergonié Institute; Jean-Michel Coindre, University Victor Ségalen, Bordeaux; Sébastien Salas, Timone Hospital, Marseille; Armelle Dufresne, Jean-Yves Blay, and Dominique Ranchere-Vince, Leon Berard Center, Lyon; Philippe Terrier, Sylvie Bonvalot, Axel Le Cesne and Odile Oberlin, Gustave Roussy Institute,
| | - Jean-Yves Blay
- Sébastien Salas, L'Institut National de la Santé et de la Recherche Médicale (INSERM) U916, Bergonié Institute, Bordeaux; Binh Bui, Eberhard Stoeckle, Véronique Brouste, and Jean Michel Coindre, Bergonié Institute; Jean-Michel Coindre, University Victor Ségalen, Bordeaux; Sébastien Salas, Timone Hospital, Marseille; Armelle Dufresne, Jean-Yves Blay, and Dominique Ranchere-Vince, Leon Berard Center, Lyon; Philippe Terrier, Sylvie Bonvalot, Axel Le Cesne and Odile Oberlin, Gustave Roussy Institute,
| | - Philippe Terrier
- Sébastien Salas, L'Institut National de la Santé et de la Recherche Médicale (INSERM) U916, Bergonié Institute, Bordeaux; Binh Bui, Eberhard Stoeckle, Véronique Brouste, and Jean Michel Coindre, Bergonié Institute; Jean-Michel Coindre, University Victor Ségalen, Bordeaux; Sébastien Salas, Timone Hospital, Marseille; Armelle Dufresne, Jean-Yves Blay, and Dominique Ranchere-Vince, Leon Berard Center, Lyon; Philippe Terrier, Sylvie Bonvalot, Axel Le Cesne and Odile Oberlin, Gustave Roussy Institute,
| | - Dominique Ranchere-Vince
- Sébastien Salas, L'Institut National de la Santé et de la Recherche Médicale (INSERM) U916, Bergonié Institute, Bordeaux; Binh Bui, Eberhard Stoeckle, Véronique Brouste, and Jean Michel Coindre, Bergonié Institute; Jean-Michel Coindre, University Victor Ségalen, Bordeaux; Sébastien Salas, Timone Hospital, Marseille; Armelle Dufresne, Jean-Yves Blay, and Dominique Ranchere-Vince, Leon Berard Center, Lyon; Philippe Terrier, Sylvie Bonvalot, Axel Le Cesne and Odile Oberlin, Gustave Roussy Institute,
| | - Sylvie Bonvalot
- Sébastien Salas, L'Institut National de la Santé et de la Recherche Médicale (INSERM) U916, Bergonié Institute, Bordeaux; Binh Bui, Eberhard Stoeckle, Véronique Brouste, and Jean Michel Coindre, Bergonié Institute; Jean-Michel Coindre, University Victor Ségalen, Bordeaux; Sébastien Salas, Timone Hospital, Marseille; Armelle Dufresne, Jean-Yves Blay, and Dominique Ranchere-Vince, Leon Berard Center, Lyon; Philippe Terrier, Sylvie Bonvalot, Axel Le Cesne and Odile Oberlin, Gustave Roussy Institute,
| | - Eberhard Stoeckle
- Sébastien Salas, L'Institut National de la Santé et de la Recherche Médicale (INSERM) U916, Bergonié Institute, Bordeaux; Binh Bui, Eberhard Stoeckle, Véronique Brouste, and Jean Michel Coindre, Bergonié Institute; Jean-Michel Coindre, University Victor Ségalen, Bordeaux; Sébastien Salas, Timone Hospital, Marseille; Armelle Dufresne, Jean-Yves Blay, and Dominique Ranchere-Vince, Leon Berard Center, Lyon; Philippe Terrier, Sylvie Bonvalot, Axel Le Cesne and Odile Oberlin, Gustave Roussy Institute,
| | - Louis Guillou
- Sébastien Salas, L'Institut National de la Santé et de la Recherche Médicale (INSERM) U916, Bergonié Institute, Bordeaux; Binh Bui, Eberhard Stoeckle, Véronique Brouste, and Jean Michel Coindre, Bergonié Institute; Jean-Michel Coindre, University Victor Ségalen, Bordeaux; Sébastien Salas, Timone Hospital, Marseille; Armelle Dufresne, Jean-Yves Blay, and Dominique Ranchere-Vince, Leon Berard Center, Lyon; Philippe Terrier, Sylvie Bonvalot, Axel Le Cesne and Odile Oberlin, Gustave Roussy Institute,
| | - Axel Le Cesne
- Sébastien Salas, L'Institut National de la Santé et de la Recherche Médicale (INSERM) U916, Bergonié Institute, Bordeaux; Binh Bui, Eberhard Stoeckle, Véronique Brouste, and Jean Michel Coindre, Bergonié Institute; Jean-Michel Coindre, University Victor Ségalen, Bordeaux; Sébastien Salas, Timone Hospital, Marseille; Armelle Dufresne, Jean-Yves Blay, and Dominique Ranchere-Vince, Leon Berard Center, Lyon; Philippe Terrier, Sylvie Bonvalot, Axel Le Cesne and Odile Oberlin, Gustave Roussy Institute,
| | - Odile Oberlin
- Sébastien Salas, L'Institut National de la Santé et de la Recherche Médicale (INSERM) U916, Bergonié Institute, Bordeaux; Binh Bui, Eberhard Stoeckle, Véronique Brouste, and Jean Michel Coindre, Bergonié Institute; Jean-Michel Coindre, University Victor Ségalen, Bordeaux; Sébastien Salas, Timone Hospital, Marseille; Armelle Dufresne, Jean-Yves Blay, and Dominique Ranchere-Vince, Leon Berard Center, Lyon; Philippe Terrier, Sylvie Bonvalot, Axel Le Cesne and Odile Oberlin, Gustave Roussy Institute,
| | - Véronique Brouste
- Sébastien Salas, L'Institut National de la Santé et de la Recherche Médicale (INSERM) U916, Bergonié Institute, Bordeaux; Binh Bui, Eberhard Stoeckle, Véronique Brouste, and Jean Michel Coindre, Bergonié Institute; Jean-Michel Coindre, University Victor Ségalen, Bordeaux; Sébastien Salas, Timone Hospital, Marseille; Armelle Dufresne, Jean-Yves Blay, and Dominique Ranchere-Vince, Leon Berard Center, Lyon; Philippe Terrier, Sylvie Bonvalot, Axel Le Cesne and Odile Oberlin, Gustave Roussy Institute,
| | - Jean-Michel Coindre
- Sébastien Salas, L'Institut National de la Santé et de la Recherche Médicale (INSERM) U916, Bergonié Institute, Bordeaux; Binh Bui, Eberhard Stoeckle, Véronique Brouste, and Jean Michel Coindre, Bergonié Institute; Jean-Michel Coindre, University Victor Ségalen, Bordeaux; Sébastien Salas, Timone Hospital, Marseille; Armelle Dufresne, Jean-Yves Blay, and Dominique Ranchere-Vince, Leon Berard Center, Lyon; Philippe Terrier, Sylvie Bonvalot, Axel Le Cesne and Odile Oberlin, Gustave Roussy Institute,
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23
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Min R, Zun Z, Lizheng W, Minjun D, Shengwen L, Wenjun Y, Chenping Z. Oral and maxillofacial desmoid-type fibromatoses in an eastern Chinese population: a report of 20 cases. ACTA ACUST UNITED AC 2011; 111:340-5. [PMID: 21247785 DOI: 10.1016/j.tripleo.2010.10.019] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/13/2010] [Revised: 09/26/2010] [Accepted: 10/13/2010] [Indexed: 11/29/2022]
Abstract
OBJECTIVE Primary desmoid-type fibromatoses arising from the oral and maxillofacial region are uncommon and hold the character of local invasion and regional recurrence. The purpose of this study was to analyze the clinicoepidemiologic characteristics of this rare disease in an eastern Chinese population. STUDY DESIGN Epidemiological and clinicopathological data on 20 patients were reviewed from the hospital records and analyzed accordingly. RESULTS Twelve males and 8 females with a ratio of 3:2 were involved. The mandible was the most common site of presentation. Suspected malignant change was found in 6 cases and an extremely high recurrence rate was also found in up to 53%. Abnormally high blood levels of alkaline phosphatase were found in 65% of patients, which had a close relation with tumor malignant change. CONCLUSION Chinese people are more susceptible to desmoid-type fibromatoses and are at an extremely high risk of regional malignant change. Elevated serum alkaline phosphatase levels may be taken as an indicator for malignant transformation.
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Affiliation(s)
- Ruan Min
- Department of Oral & Maxillofacial Surgery, Ninth People's Hospital, Shanghai Jiao Tong University, School of Medicine, Shanghai Key Laboratory of Stomatology, Shanghai, P R China
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