Primary mediastinal synovial sarcoma: a case report and review of the literature

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Abstract

Primary mediastinal synovial sarcoma is a rare malignancy with only a few cases reported so far. A 56-year-old woman was admitted to our hospital for an investigation of a nodule in the left middle lung on chest radiography. Computed tomography revealed a mediastinal mass first described as a solitary fibrous tumor. The diagnosis of synovial sarcoma was established by computed tomography-guided percutaneous needle biopsy. Work up showed no metastasis to distant organs or contralateral pleural cavity. The mass was surgically resected; pathological and immunohistochemical analyses confirmed the diagnosis of a monophasic spindle cell synovial sarcoma probably originating from phrenic nerve. The patient received adjuvant chemotherapy and radiation and is free of recurrence after a follow up of 16 months.

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The evolving classification of soft tissue tumours: an update based on the new WHO classification.

Christopher Fletcher (2005)

Tumour classifications have become an integral part of modern oncology and, for pathologists, they provide guidelines which facilitate diagnostic and prognostic reproducibility. In many organ systems and most especially over the past decade or so, the World Health Organization (WHO) classifications have become pre-eminent, partly enabled by the timely publication of new "blue books" which now incorporate detailed text and copious illustrations. The new WHO classification of soft tissue tumours was introduced in late 2002 and, because it represents a broad consensus view, it has gained widespread acceptance. This review summarizes the changes, both major and minor, which were introduced and briefly describes the significant number of tumour types which have been first recognized or properly characterized during the past decade. Arguably the four most significant conceptual advances have been: (i) the formal recognition that morphologically benign lesions (such as cutaneous fibrous histiocytoma) may very rarely metastasize; (ii) the general acceptance that most pleomorphic sarcomas can be meaningfully subclassified and that so-called malignant fibrous histiocytoma is not a definable entity, but instead represents a wastebasket of undifferentiated pleomorphic sarcomas, accounting for no more than 5% of adult soft tissue sarcomas; (iii) the acknowledgement that most lesions formerly known as haemangiopericytoma show no evidence of pericytic differentiation and, instead, are fibroblastic in nature and form a morphological continuum with solitary fibrous tumour; and (iv) the increasing appreciation that not only do we not know from which cell type(s) most soft tissue tumours originate (histogenesis) but, for many, we do not recognize their line of differentiation or lineage--hence an increasing number of tumours are placed in the "uncertain differentiation" category.

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Synovial sarcoma: a retrospective analysis of 271 patients of all ages treated at a single institution.

Andrea Ferrari, Alessandro Gronchi, Michela Casanova … (2004)

The optimal treatment strategy for synovial sarcoma (SS) is subject to debate, and different strategies have been used for pediatric and adult patients. The current retrospective analysis examined a large group of patients of all ages who were treated at a single institution over a 30-year period. The study included 271 patients who ranged in age from 5 years to 87 years; 255 had localized disease, which was macroscopically resected in 215 cases and deemed unresectable at diagnosis in 40 cases. Chemotherapy was administered to 41% of patients, corresponding to 76% of patients age or 16 years and 30 years, respectively). Chemotherapy was used more commonly for children than for adults. Among patients with surgically resected disease, the 5-year metastasis-free survival (MFS) rate was 60% for those who were treated with chemotherapy and 48% for those who were not; the benefit associated with chemotherapy use appeared to be greatest for patients age > or = 17 years who had tumors measuring > 5 cm (MFS, 47% [chemotherapy] vs. 27% [no chemotherapy]). In the subgroup of patients with measurable disease, the rate of tumor response to chemotherapy was approximately 48%. Although the authors await more convincing proof of the efficacy of adjuvant chemotherapy in the treatment of adult soft tissue sarcoma, they recommend that patients with high-risk SS (tumor size > 5 cm) be the first to be considered for this type of treatment.

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Synovial sarcoma: a clinicopathologic, staging, and prognostic assessment.

A Spillane, R A'Hern, I R Judson … (2000)

Synovial sarcoma (SS) is a common soft tissue sarcoma (STS) with a propensity for young adults and notable sensitivity to chemotherapy (CT). This study provides a current clinicopathologic, staging, and prognostic assessment for SS. The problems with the current American Joint Committee for Cancer (AJCC) Staging System in relation to SS are discussed. Review of a prospective database supplemented by retrospective data. One hundred fifty patients were assessed; median age was 30 years and median follow-up was 52 months. Overall actuarial 5-year survival rate was 57%. Size trend, but not a cutoff of less than 5 cm versus > or = 5 cm, was a prognostic indicator (P <.001). The current AJCC/International Union Against Cancer Staging System differentiated prognosis less well than the recently proposed Royal Marsden Hospital Staging System. Age greater than 20 years at diagnosis implied worse prognosis. A local recurrence event was associated with a worse survival (P <.001). Therapeutic CT was administered to 55 patients. Eleven of 19 patients had an objective response to a combination of ifosfamide and doxorubicin. Four cases had complete response after CT. Twenty-one patients had pulmonary metastasectomy, with an actuarial 5-year survival rate of 23%. SS tends to affect young people. In this subtype of STS, size trend is the most significant influence on stage and hence survival; however, smaller SSs have an unexpectedly poor prognosis. Adequate local control may affect survival. SS is often chemosensitive, and given its poor prognosis, multicenter trials of adjuvant therapy are warranted.

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Author and article information

Journal

Journal ID (nlm-ta): Cases J

Title: Cases Journal

Publisher: Cases Network Ltd

ISSN (Electronic): 1757-1626

Publication date (Electronic): 07 August 2009

Publication date Collection: 2009

Volume: 2

Electronic Location Identifier: 6948

Affiliations

[1 ]simpleDepartment of Radiology, Innsbruck Medical University Anichstraße 35, 6020 InnsbruckAustria

[2 ]simpleInstitute of Pathology, Innsbruck Medical University Anichstraße 35, 6020 InnsbruckAustria

[3 ]simpleDepartment of Nuclear Medicine, Innsbruck Medical University Anichstraße 35, 6020 InnsbruckAustria

[4 ]simpleDepartment of Surgery, University of Virginia Health Services Charlottesville, VAUSA

[5 ]simpleDepartment of Cardiac Surgery, Innsbruck Medical University Anichstraße 35, 6020 InnsbruckAustria

[6 ]simpleDepartment of Oncology, Natters Hospital In der Stille 20, 6161 NattersAustria

[7 ]simpleDepartment of Pulmonology, Natters Hospital In der Stille 20, 6161 NattersAustria

Article

Publisher ID: 6948

DOI: 10.4076/1757-1626-2-6948

PMC ID: 2769329

PubMed ID: 19918499

SO-VID: b2bbe5d3-77b0-4d58-91b5-37dd7b5ef3ad

License:

This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

History

Date received : 27 March 2009

Date accepted : 09 July 2009

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Primary mediastinal synovial sarcoma: a case report and review of the literature

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Most cited references 16

The evolving classification of soft tissue tumours: an update based on the new WHO classification.

Synovial sarcoma: a retrospective analysis of 271 patients of all ages treated at a single institution.

Synovial sarcoma: a clinicopathologic, staging, and prognostic assessment.

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