|
1
|
Chen JY, Cen B, Hu F, Qiu Y, Xiao GM, Zhou JG, Zhang FC. Clinical characteristics and outcomes of primary intracranial alveolar soft-part sarcoma: A case report. World J Clin Cases 2022; 10:296-303. [PMID: 35071531 PMCID: PMC8727262 DOI: 10.12998/wjcc.v10.i1.296] [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: 05/18/2021] [Revised: 07/15/2021] [Accepted: 11/30/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Primary intracranial alveolar soft-part sarcoma (PIASPS) is a rare malignancy. We aimed to investigate the clinical profiles and outcomes for PIASPS.
CASE SUMMARY We firstly reported five consecutive cases from our institute. Then, the cases from previous studies were pooled and analyzed to delineate the characteristics of this disease. Our cohort included two males and three females. The median age was 21-years-old (range: 8-54-years-old). All the patients received surgical treatment. Gross total resection (GTR), radiotherapy, and chemotherapy were administered in 3 patients, 4 patients, and 1 patient, respectively. After a median follow-up of 36 mo, tumor progression was noticed in 4 patients; and 3 patients died of the disease. Pooled data (n = 14) contained 5 males and 9 females with a median age of 19 years. The log-rank tests showed that GTR (P = 0.011) could prolong progression-free survival, and radiotherapy (P < 0.001) resulted in longer overall survival.
CONCLUSION Patients with PIASPS suffer from poor outcomes. Surgical treatment is the first choice, and GTR should be achieved when the tumor is feasible. Patients with PIASPS benefit from radiotherapy, which should be considered as a part of treatment therapies.
Collapse
Affiliation(s)
- Jun-Yu Chen
- Department of Neurosurgery, General Hospital of the Yangtze River Shipping, Wuhan 430010, Hubei Province, China
| | - Bo Cen
- Department of Neurosurgery, General Hospital of the Yangtze River Shipping, Wuhan 430010, Hubei Province, China
| | - Fei Hu
- Department of Neurosurgery, General Hospital of the Yangtze River Shipping, Wuhan 430010, Hubei Province, China
| | - Yong Qiu
- Department of Neurosurgery, General Hospital of the Yangtze River Shipping, Wuhan 430010, Hubei Province, China
| | - Guo-Min Xiao
- Department of Neurosurgery, General Hospital of the Yangtze River Shipping, Wuhan 430010, Hubei Province, China
| | - Jun-Ge Zhou
- Department of Neurosurgery, General Hospital of the Yangtze River Shipping, Wuhan 430010, Hubei Province, China
| | - Fang-Cheng Zhang
- Department of Neurosurgery, Wuhan Union Hospital, Wuhan 430010, Hubei Province, China
| |
Collapse
|
|
2
|
Damante MA, Huntoon KM, Palmer JD, Liebner DA, Elder JB. A case of multiple synchronously diagnosed brain metastases from alveolar soft part sarcoma without concurrent lung involvement. Surg Neurol Int 2021; 12:111. [PMID: 33880216 PMCID: PMC8053428 DOI: 10.25259/sni_554_2020] [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: 08/19/2020] [Accepted: 02/15/2021] [Indexed: 11/13/2022] Open
Abstract
Background: Alveolar soft part sarcoma (ASPS) is a rare soft-tissue sarcoma with a propensity for early hematogenous dissemination to the lungs and frequent brain metastasis. The development of lung metastasis almost invariably precedes intracranial involvement. There are no previously reported cases in which a patient was synchronously diagnosed with ASPS and multiple brain metastasis without lung involvement. Case Description: A 29-year-old gentleman was found to have three intracranial lesions following the onset of generalized seizures. Staging studies identified a soft-tissue mass in the left thigh and an adjacent femoral lesion. Biopsy of the soft-tissue mass was consistent with ASPS. The patient then underwent neoadjuvant stereotactic radiotherapy to all three brain lesions, followed by en bloc resection of the dominant lesion. The patient was then started on a programmed death-ligand 1 (PD-L1) inhibitor. Subsequent surgical resection of the primary lesion and femur metastasis demonstrates a histopathologic complete response of the bony metastasis and partial response of the primary lesion. At present, the patient has received 14 cycles of atezolizumab without recurrence of the primary or bony lesions and the irradiated intracranial disease has remained stable without recurrence of the resected dominant lesion. Conclusion: While intracranial involvement is relatively common in ASPS, a case with multiple, synchronously diagnosed brain metastasis without concurrent lung metastasis has not been described. The presented case discusses the safety and efficacy of aggressive management of intracranial disease in the setting of atezolizumab. Prospective evaluation of the efficacy of checkpoint inhibitors and the prognostic value of PD-L1 expression in ASPS with brain metastasis are necessary.
Collapse
Affiliation(s)
- Mark A Damante
- Department of Neurosurgery, The Ohio State University Wexner Medical Center, Columbus, Ohio, United States
| | - Kristin M Huntoon
- Department of Neurosurgery, The Ohio State University Wexner Medical Center, Columbus, Ohio, United States
| | - Joshua D Palmer
- Department of Neurosurgery, The Ohio State University Wexner Medical Center, Columbus, Ohio, United States.,Department of Radiation Oncology, The Ohio State University Wexner Medical Center, Columbus, Ohio, United States
| | - David A Liebner
- Department of Internal Medicine, The Ohio State University Wexner Medical Center, Columbus, Ohio, United States
| | - James Bradley Elder
- Department of Neurosurgery, The Ohio State University Wexner Medical Center, Columbus, Ohio, United States
| |
Collapse
|
|
3
|
Stereotactic radiosurgery as a primary treatment for metastatic skull base alveolar soft part sarcoma: a case report. Acta Neurochir (Wien) 2021; 163:351-355. [PMID: 32686069 DOI: 10.1007/s00701-020-04496-2] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/15/2020] [Accepted: 07/14/2020] [Indexed: 10/23/2022]
Abstract
Alveolar soft part sarcoma (ASPS) is a rare malignancy that typically arises in the trunk or extremities and preferentially metastasises to the brain. Radical resection is generally recommended for cranial metastatic ASPS, but stereotactic radiosurgery (SRS) is a recognised alternative for tumours in surgically challenging locations. Here, we present the case of a 22-year-old female, who underwent SRS and systemic therapy with pazopanib for a metastatic ASPS in the left temporal bone. The tumour was successfully controlled without further intervention over 23 months following SRS, which should be considered for metastatic ASPS when surgical resection is not appropriate.
Collapse
|
|
4
|
Alveolar soft part sarcoma: the new primary intracranial malignancy : A case report and review of the literature. Neurosurg Rev 2017; 42:23-29. [PMID: 28744773 PMCID: PMC6394700 DOI: 10.1007/s10143-017-0874-4] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/07/2017] [Accepted: 06/22/2017] [Indexed: 01/21/2023]
Abstract
The purpose of this paper is to serve as a reference to aid in the management of this poorly understood intracranial malignancy. The authors report their experience treating the eighth ostensible case of a primary intracranial alveolar soft part sarcoma (ASPS). A 21-year-old man presented to hospital after collapsing. He gave a 1-year history of headache, a 2-month history of reduced visual acuity and on examination had left facial paraesthesia with left-sided incoordination. MRI of the brain revealed a large left posterior fossa mass. The patient underwent resection of the tumour with good recovery in function. Immunohistochemical analysis of the tumour specimen confirmed an ASPS, and multimodal imaging in search of an extra-cranial disease primary was negative. A review of the literature yielded only seven other cases of primary intracranial ASPS. A variety of diagnostic imaging modalities were employed in search of a disease primary, as were various combinations of surgical resection, chemotherapy and radiotherapy as treatment. Half of the cases documented delayed disease recurrence. The authors discuss the following: the unique radiological and immunohistological characteristics of this disease including the potential for its misdiagnosis; the investigations required to diagnose a primary intracranial ASPS; the efficacy of current medical and surgical treatment options and the factors that will aid in prognostication. This is the first review of this new primary intracranial malignancy. From our analysis, we offer a joint radiological and immunohistochemical algorithm for the diagnosis of primary intracranial ASPS and specific operative considerations prior to resection.
Collapse
|
|
5
|
Abstract
PURPOSE OF REVIEW Survival rates for children with metastatic sarcoma have remained dismal despite intensified multiagent chemotherapy protocols. The local treatment of metastatic disease has been promoted as a way to eliminate colonies of genetically unstable, heterogeneous metastatic cells in an attempt to improve survival amongst this most unfortunate patient population. RECENT FINDINGS The survival benefit offered by pulmonary metastasectomies in patients with metastatic osteosarcoma is well substantiated. Utilization of other local treatment modalities, such as radiation therapy and percutaneous thermal ablation, offers the opportunity to intervene in a wide range of pulmonary and extrapulmonary metastatic disease. Patients who have the entirety of their identifiable disease addressed by local control modalities consistently demonstrate improved survival compared with patients who are treated with systemic therapy in isolation. SUMMARY The current state of the literature prevents a definitive conclusion about the utility of local control for metastatic sarcoma. The retrospective trials are clouded by selection bias and the prospective studies are designed to address alternative questions. However, the techniques utilized for local control impart minimal risk to the patient and, in amenable cases, have been shown to provide an opportunity to effect a cure in children with an otherwise dismal prognosis.
Collapse
|
|
6
|
Shweikeh F, Bukavina L, Saeed K, Sarkis R, Suneja A, Sweiss F, Drazin D. Brain metastasis in bone and soft tissue cancers: a review of incidence, interventions, and outcomes. Sarcoma 2014; 2014:475175. [PMID: 24757391 PMCID: PMC3976890 DOI: 10.1155/2014/475175] [Citation(s) in RCA: 58] [Impact Index Per Article: 5.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/30/2013] [Revised: 01/19/2014] [Accepted: 01/22/2014] [Indexed: 12/15/2022] Open
Abstract
Bone and soft tissue malignancies account for a small portion of brain metastases. In this review, we characterize their incidence, treatments, and prognosis. Most of the data in the literature is based on case reports and small case series. Less than 5% of brain metastases are from bone and soft tissue sarcomas, occurring most commonly in Ewing's sarcoma, malignant fibrous tumors, and osteosarcoma. Mean interval from initial cancer diagnosis to brain metastasis is in the range of 20-30 months, with most being detected before 24 months (osteosarcoma, Ewing sarcoma, chordoma, angiosarcoma, and rhabdomyosarcoma), some at 24-36 months (malignant fibrous tumors, malignant peripheral nerve sheath tumors, and alveolar soft part sarcoma), and a few after 36 months (chondrosarcoma and liposarcoma). Overall mean survival ranges between 7 and 16 months, with the majority surviving < 12 months (Ewing's sarcoma, liposarcoma, malignant fibrous tumors, malignant peripheral nerve sheath tumors, angiosarcoma and chordomas). Management is heterogeneous involving surgery, radiosurgery, radiotherapy, and chemotherapy. While a survival advantage may exist for those given aggressive treatment involving surgical resection, such patients tended to have a favorable preoperative performance status and minimal systemic disease.
Collapse
Affiliation(s)
- Faris Shweikeh
- College of Medicine, Northeast Ohio Medical University, 4209 State Route 44, Rootstown, OH 44272, USA
| | - Laura Bukavina
- Department of Medicine, Summa Health System, Akron, OH 44303, USA
- Johns Hopkins School of Public Health, Baltimore, MD 21205, USA
| | - Kashif Saeed
- Department of Physical Medicine and Rehabilitation, Rush University Medical Center, Chicago, IL 60612, USA
| | - Reem Sarkis
- College of Medicine, Northeast Ohio Medical University, 4209 State Route 44, Rootstown, OH 44272, USA
| | - Aarushi Suneja
- College of Medicine, Northeast Ohio Medical University, 4209 State Route 44, Rootstown, OH 44272, USA
| | - Fadi Sweiss
- Department of Neurosurgery, George Washington University, Washington, DC 20037, USA
| | - Doniel Drazin
- Department of Neurosurgery, Cedars-Sinai Medical Center, Los Angeles, CA 90048, USA
| |
Collapse
|
|
7
|
Sabel MS, Gibbs JF, Litwin A, McGrath B, Kraybill WB, Brooks JJ. Alveolar soft part sarcoma metastatic to small bowel mucosa causing polyposis and intussuseption. Sarcoma 2011; 5:133-7. [PMID: 18521439 PMCID: PMC2408368 DOI: 10.1080/13577140120048584] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/15/2022] Open
Abstract
A report of alveolar soft part sarcoma metastatic to the small bowel is presented. Hematogenous metastases to the small
bowel from primary tumors outside the abdominal cavity are uncommon, and most remain asymptomatic and are not discovered
until autopsy. However, small bowel metastases can lead to intestinal obstruction, intussuseption or even perforation.
While metastases to the small bowel have been described for other tumor types, including melanoma and lung cancer, this
is extremely uncommon for sarcoma, especially alveolar soft part sarcoma. We describe a 42-year-old male with a long history
of alveolar soft part sarcoma, metastatic to the lung and brain, who developed an intussuseption from metastases to the small
bowel.
Collapse
Affiliation(s)
- M S Sabel
- Department of Surgical Oncology Roswell Park Cancer Institute and State University of Buffalo at New York Buffalo New York USA
| | | | | | | | | | | |
Collapse
|
|
8
|
Salvati M, D'Elia A, Frati A, Santoro A. Sarcoma metastatic to the brain: a series of 35 cases and considerations from 27 years of experience. J Neurooncol 2009; 98:373-7. [PMID: 20039192 DOI: 10.1007/s11060-009-0085-0] [Citation(s) in RCA: 40] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/24/2009] [Accepted: 11/30/2009] [Indexed: 10/20/2022]
Abstract
The authors report their 27-year experience regarding 35 cases of supratentorial brain metastasis from sarcoma treated in a single institution: these included ten osteosarcomas, seven leiomyosarcomas, five Ewing sarcomas, four malignant fibrous histiocytomas, three alveolar soft-part sarcomas (ASPS), two rhabdomyosarcomas, one liposarcoma, and three unclassified sarcomas. The first 15 cases of the series have already been described in a previous publication. Median survival after craniotomy was 9.8 months (range: 4-24). In patients with preoperative Karnofsky performance score (KPS) > 60 it was 12.8 months (range: 6.5-24 months) versus 5.4 months for those patients with a KPS < or = 60 (P = 0.01). Eight patients had more than one lesion, six of which were treated in the last ten years. Of the three patients with ASPS, the first two were alive at 15 and 20 months (before being lost to follow-up) whereas the third patient is alive at 24 month follow-up. The authors conclude that surgery is more effective in treating selected patients with sarcoma metastatic to the brain, and that patients with metastasis from ASPS have good prognosis when submitted to surgical treatment. The complete removal of all brain metastases "en bloc" and a KPS > 60 are associated with the best prognosis. Finally, it seems that surgical indications for multiple brain metastases from sarcoma have increased during the last ten years.
Collapse
Affiliation(s)
- M Salvati
- Neuroscience-Neurosurgery Department, "Sapienza" University, Rome, Italy.
| | | | | | | |
Collapse
|
|
9
|
Daigeler A, Kuhnen C, Hauser J, Goertz O, Tilkorn D, Steinstraesser L, Steinau HU, Lehnhardt M. Alveolar soft part sarcoma: clinicopathological findings in a series of 11 cases. World J Surg Oncol 2008; 6:71. [PMID: 18593459 PMCID: PMC2459182 DOI: 10.1186/1477-7819-6-71] [Citation(s) in RCA: 34] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/25/2008] [Accepted: 07/01/2008] [Indexed: 12/13/2022] Open
Abstract
Background Alveolar sarcoma of the soft parts (ASPS) represents a very rare entity of soft tissue sarcoma with special features such as young peak age incidence and frequent metastasis to the brain. The aim of this study was a clinicopathological analysis with special reference to treatment and outcome. Methods From the database of the BG-University Hospital Bergmannsheil, 1597 soft tissue sarcoma (STS) cases were reviewed and 11 consecutive patients with ASPS were isolated. Data was acquired from patients' charts and contact to patients, their relatives or general practitioners, with special reference to treatment and clinical course. The average follow up time from the time of the definite operation for the primary tumor was 6.5 years. Kaplan-Meier method was used to calculate survival. Results Patients with localized disease who received complete resection and adjuvant radiation and who did not develop recurrence or metastatic disease within 2 years after surgery had a positive outcome. The size of the tumor, its localization, and the time of untreated growth before treatment did not influence the long-term results. All patients who developed recurrent disease also suffered from distant metastasis, reflecting the aggressive biology of the tumor. All patients with distant metastasis had the lungs and the brain affected. Conclusion Due to the limited number of patients with ASPS, prospective studies would have to span decades to gather a significant collective of patients; therefore, it is not possible to comment meaningfully on a possible benefit of neoadjuvant or adjuvant therapy. We recommend wide surgical excision and, in the absence of data telling otherwise, adjuvant radiation. In cases with recurrent disease or metastasis, the prognosis is bad and further treatment will be restricted to palliation in most cases.
Collapse
Affiliation(s)
- Adrien Daigeler
- Department of Plastic Surgery, Burn Center, Hand surgery, Sarcoma Reference Center, BG-University Hospital Bergmannsheil, Ruhr University Bochum, Buerkle-de-la-Camp-Platz 1, 44789 Bochum, Germany.
| | | | | | | | | | | | | | | |
Collapse
|
|
10
|
Bu X, Bernstein L. A proposed explanation for female predominance in alveolar soft part sarcoma. Cancer 2005; 103:1245-53. [PMID: 15693033 DOI: 10.1002/cncr.20899] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/08/2022]
Abstract
BACKGROUND Alveolar soft-part sarcoma (ASPS) is a rare malignant soft tissue tumor with both clinically and morphologically distinct features. It often involves the extremities of adolescents and young adults and shows a predilection for females. Recently, ASPS was found to have a nonreciprocal der(17)t(X;17) translocation with the corresponding fusion gene located in chromosome 17. Because females have an extra X-chromosome, their likelihood of developing an X;autosome translocation is theoretically double that of males, and thus, this extra X-chromosome is a likely explanation for female predominance of ASPS. METHODS The authors used data from the National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) registry program, which included 87 ASPS cases (33 males and 54 females), and published ASPS cases, which included 317 cases (121 males and 196 females), to test our hypothesis. The authors compared the observed proportion of female cases with that expected under the two X-chromosomes-double-risk hypothesis including the consideration of X-inactivation status. RESULTS The hypothesis that the fusion gene is not subject to X-inactivation is supported by data (P = 0.6, 0.24, and 0.20 for SEER cases, published cases, and their combination, respectively). In contrast, the competing hypothesis that the fusion gene is subject to X-inactivation is rejected (P = 0.007, < 0.00001, and < 0.00001 for SEER cases, published cases, and their combination, respectively). CONCLUSIONS Therefore, the authors found a statistical association between the female predominance observed in ASPS and female possession of an extra X-chromosome/noninactivation of the ASPS X;autosome translocation fusion gene.
Collapse
Affiliation(s)
- Xiangdong Bu
- Department of Pathology, Keck School of Medicine, University of Southern California, Los Angeles, California, USA.
| | | |
Collapse
|
|
11
|
Reichardt P, Lindner T, Pink D, Thuss-Patience PC, Kretzschmar A, Dörken B. Chemotherapy in alveolar soft part sarcomas. What do we know? Eur J Cancer 2003; 39:1511-6. [PMID: 12855256 DOI: 10.1016/s0959-8049(03)00264-8] [Citation(s) in RCA: 95] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/21/2023]
Abstract
Alveolar soft part sarcoma (ASPS) is a rare tumour. Published series about treatment and outcome are scarce. Conclusive data about the response to chemotherapy are not available. The aim of this study was to analyse the efficacy of palliative chemotherapeutic treatment options and the incidence and mode of presentation of brain metastases. We retrospectively analysed our own sarcoma data-base and reviewed the literature. From our registry containing 757 patients, we identified 8 patients with ASPS. From the literature, 47 cases of adult patients and 13 children with sufficient data about chemotherapy were identified. Response to first-line chemotherapy in 68 patients was: complete remission (CR) 4%, partial remission (PR) 3%, stable disease (SD) 41%, progressive disease (PD) 51%. 285 patients with stage IV disease were evaluable for the analysis of metastatic sites. The incidence of brain metastases was 30.5% (87/285). Brain metastases were detected at a median interval of 48 months (range 0-396 months) after the primary diagnosis. Median survival after the diagnosis of brain metastases was 12 months. The median survival for patients with stage IV disease treated by chemotherapy was 36+ months (range 10-132 months) (31 patients evaluable) with a median follow-up of 46 months (range 10-135 months). ASPS shows a high incidence of brain metastases, at least 3 times higher than that of other soft tissue sarcomas. Chemotherapeutic regimens used for the treatment of other soft tissue sarcomas lack efficacy in ASPS. Staging investigations for ASPS should routinely include imaging of the brain. ASPS patients should not be treated with chemotherapy outside of controlled clinical trials. New targets for specific biologically-directed therapies need to be developed.
Collapse
Affiliation(s)
- P Reichardt
- Medizinische Klinik m. S. Hämatologie, Onkologie und Tumorimmunologie, Robert-Rössle-Klinik, Helios-Klinikum Berlin, Universitätsklinikum Charité, Humboldt-Universität zu Berlin, Berlin, Germany.
| | | | | | | | | | | |
Collapse
|
|
12
|
Abstract
The aim of this study was to evaluate the presentation, course and treatment outcome of 15 patients with this rare type of sarcoma. The files of the patients were retrospectively analysed. Overall survival was calculated according to the Kaplan-Meier method. There were 15 patients, 8 male and 7 female. The mean age at diagnosis was 29 years for men and 24 years for women. The median survival was 48 months with an overall 5-year survival of 38%. 5 patients had haematogenic metastases at the time of diagnosis. For the remaining 10 patients with localised disease, the median survival was 48 months and the 5-year survival 48%. The median disease-free survival for these patients was 12 months with a 5-year disease free survival of 40%. After the occurrence of haematogenic metastases, patients survived a median period of 8 months (range 0-45 months). 5 patients are still free of disease after a median period of 234 months (12-295 months). Alveolar soft part sarcoma is found especially in young adults. When diagnosed, it is often metastasised with a poor prognosis. However, when radically resected, long-term survival is possible.
Collapse
Affiliation(s)
- S van Ruth
- Department of Surgery, The Netherlands Cancer Institute/Antoni van Leeuwenhoek Hospital, Plesmanlaan 120, 1066 CX Amsterdam, The Netherlands
| | | | | | | |
Collapse
|
|
13
|
Kurtz JE, Andrès E, Rohr S, Maloisel F, Mechine A, Meyer C, Dufour P. Pancreatic metastasis of alveolar soft-part sarcoma: a case report and review of the literature. Ann Oncol 2001; 12:865-7. [PMID: 11484966 DOI: 10.1023/a:1011121230825] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/12/2022] Open
Abstract
Alveolar soft-part sarcoma is a rare tumour. Patients commonly present with distant metastases both at the time of diagnosis and late in the course of disease. We present a case of pancreatic metastasis, occurring more than six years after diagnosis. Treatment consisted in subtotal pancreatoduodenectomy with pylorus resection. Both specific patterns of relapse and treatment opportunities of this uncommon feature are discussed.
Collapse
Affiliation(s)
- J E Kurtz
- Department of Onco-Hematology, Hĵpitaux Universitaires de Strasbourg, France
| | | | | | | | | | | | | |
Collapse
|
|
14
|
Portera CA, Ho V, Patel SR, Hunt KK, Feig BW, Respondek PM, Yasko AW, Benjamin RS, Pollock RE, Pisters PW. Alveolar soft part sarcoma: clinical course and patterns of metastasis in 70 patients treated at a single institution. Cancer 2001. [PMID: 11169942 DOI: 10.1002/1097-0142(20010201)91:3%3c585::aid-cncr1038%3e3.0.co;2-0] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/10/2022]
Abstract
BACKGROUND Alveolar soft part sarcoma (ASPS) is a rare form of soft tissue sarcoma. Brain metastases have been reported to be a common feature of Stage IV ASPS, and recent practice guidelines recommend routine intracranial imaging as part of the staging evaluation in all patients who present with ASPS. METHODS The authors performed a comprehensive retrospective review of the clinical presentation, treatment, outcome, and patterns of failure in a consecutive series of patients with localized (American Joint Committee on Cancer [AJCC] Stages II/III) or metastatic (AJCC Stage IV) ASPS who presented to a tertiary care cancer center between 1959 and 1998. RESULTS Seventy-four patients were identified from the database searches. The anatomic distribution of their primary tumors included: extremities, 44 patients (60%); trunk, 15 patients (20%); head and neck, 9 patients (12%); and retroperitoneum, 6 patients (8%). The median tumor size was 6.5 cm (range, 1.2-24 cm). The AJCC stage at presentation was Stage II or III in 35% of the patients and Stage IV in 65% of the patients. The 5-year actuarial local recurrence free, distant recurrence free, disease free, and overall survival rates among the 22 patients with localized ASPS were 88%, 84%, 71%%, and 87%, respectively. At a median follow-up of 9 years, 2 of 22 patients with localized disease had developed local recurrences and 3 had developed metastatic disease (all to the lung only). Brain metastases were noted in 9 of 48 patients who presented with Stage IV (M1) disease (19%) and always were noted in association with metastasis to other sites. The median survival of patients with M1 disease was 40 months, with a 5-year survival rate of 20%. CONCLUSIONS Long term follow-up of patients with localized ASPS reveals a relatively indolent clinical course with relatively low rates of local and distant recurrence. In patients with Stage IV ASPS, brain metastases were observed only as part of more disseminated disease. The observations of the current study do not support current practice guidelines for the staging of patients with ASPS and suggest that selective rather than routine intracranial imaging should be used in patients presenting with ASPS.
Collapse
Affiliation(s)
- C A Portera
- The University of Texas M. D. Anderson Cancer Center Multidisciplinary Sarcoma Center, Houston 77030-4009, USA
| | | | | | | | | | | | | | | | | | | |
Collapse
|
|
15
|
Portera CA, Ho V, Patel SR, Hunt KK, Feig BW, Respondek PM, Yasko AW, Benjamin RS, Pollock RE, Pisters PW. Alveolar soft part sarcoma: clinical course and patterns of metastasis in 70 patients treated at a single institution. Cancer 2001; 91:585-91. [PMID: 11169942 DOI: 10.1002/1097-0142(20010201)91:3<585::aid-cncr1038>3.0.co;2-0] [Citation(s) in RCA: 231] [Impact Index Per Article: 9.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Abstract
BACKGROUND Alveolar soft part sarcoma (ASPS) is a rare form of soft tissue sarcoma. Brain metastases have been reported to be a common feature of Stage IV ASPS, and recent practice guidelines recommend routine intracranial imaging as part of the staging evaluation in all patients who present with ASPS. METHODS The authors performed a comprehensive retrospective review of the clinical presentation, treatment, outcome, and patterns of failure in a consecutive series of patients with localized (American Joint Committee on Cancer [AJCC] Stages II/III) or metastatic (AJCC Stage IV) ASPS who presented to a tertiary care cancer center between 1959 and 1998. RESULTS Seventy-four patients were identified from the database searches. The anatomic distribution of their primary tumors included: extremities, 44 patients (60%); trunk, 15 patients (20%); head and neck, 9 patients (12%); and retroperitoneum, 6 patients (8%). The median tumor size was 6.5 cm (range, 1.2-24 cm). The AJCC stage at presentation was Stage II or III in 35% of the patients and Stage IV in 65% of the patients. The 5-year actuarial local recurrence free, distant recurrence free, disease free, and overall survival rates among the 22 patients with localized ASPS were 88%, 84%, 71%%, and 87%, respectively. At a median follow-up of 9 years, 2 of 22 patients with localized disease had developed local recurrences and 3 had developed metastatic disease (all to the lung only). Brain metastases were noted in 9 of 48 patients who presented with Stage IV (M1) disease (19%) and always were noted in association with metastasis to other sites. The median survival of patients with M1 disease was 40 months, with a 5-year survival rate of 20%. CONCLUSIONS Long term follow-up of patients with localized ASPS reveals a relatively indolent clinical course with relatively low rates of local and distant recurrence. In patients with Stage IV ASPS, brain metastases were observed only as part of more disseminated disease. The observations of the current study do not support current practice guidelines for the staging of patients with ASPS and suggest that selective rather than routine intracranial imaging should be used in patients presenting with ASPS.
Collapse
Affiliation(s)
- C A Portera
- The University of Texas M. D. Anderson Cancer Center Multidisciplinary Sarcoma Center, Houston 77030-4009, USA
| | | | | | | | | | | | | | | | | | | |
Collapse
|
|
16
|
Bouffet E, Doumi N, Thiesse P, Mottolese C, Jouvet A, Lacroze M, Carrie C, Frappaz D, Brunat-Mentigny M. Brain metastases in children with solid tumors. Cancer 1997; 79:403-10. [PMID: 9010115 DOI: 10.1002/(sici)1097-0142(19970115)79:2<403::aid-cncr25>3.0.co;2-3] [Citation(s) in RCA: 63] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/03/2023]
Abstract
BACKGROUND Brain metastases are uncommon among children with solid tumors. However, improvements in survival have increased the period of time during which children are at risk for developing these metastases. The authors reviewed brain metastases in children with solid tumors treated at the Centre Léon Bérard during the 9 years between 1987 and 1995. METHODS Among 486 patients with solid tumors, 162 eventually developed distant metastases in their disease process, including 12 brain metastases detected by imaging. The tumor type, clinical setting, imaging characteristics, treatment modalities, and outcome were assessed for each patient. RESULTS The most common tumors causing brain metastases were Ewing's sarcoma (in three patients), neuroblastoma (in three patients), and osteogenic sarcoma (in three patients). At the time of initial diagnosis, 9 of the 12 patients had metastatic disease. All but one patient initially received intensive multiagent chemotherapy, including high dose chemotherapy with bone marrow rescue in six patients. The median time from initial diagnosis to the detection of brain metastases was 15 months. These metastases were clinically detectable in 10 patients and subclinical in 2 patients. Brain metastases were present at the time of first relapse in five patients. In two patients, the brain was the only site of relapse. All other patients had extensive systemic disease. Seven patients had multiple brain metastases. Two children underwent surgical resection of solitary metastases, and eight were irradiated. One child achieved complete remission following chemotherapy and irradiation. All other children died, mostly of their systemic disease, within a median period of 3 months. CONCLUSIONS The introduction of effective systemic chemotherapy has changed the patterns of brain metastases in children. The increasing incidence of these metastases in patients with sarcoma and neuroblastoma suggests that the brain is a pharmacologically protected site in patients initially diagnosed with metastatic disease.
Collapse
Affiliation(s)
- E Bouffet
- Department of Pediatric Oncology, Centre Léon Bérard, Lyon, France
| | | | | | | | | | | | | | | | | |
Collapse
|