Primary Clear-Cell Sarcoma in the Mediastinum

Tanaka, Yumi; Yoshimasu, Tatsuya; Oura, Shoji; Hirai, Yoshimitsu; Kawago, Mitsumasa; Okamura, Yoshitaka

doi:10.1159/000363180

Cited by 4 publications

(4 citation statements)

References 7 publications

(13 reference statements)

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“…Two cases of clear cell sarcoma (CCS; melanoma of soft parts) have been reported as primary tumours in the mediastinum [ 194 , 199 ]. A 15-cm tumour was excised from the superior mediastinum of a 59-year-old female in the report by Tirabosco et al [ 199 ].…”

Section: Tumours Of Uncertain Differentiationmentioning

confidence: 99%

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Mesenchymal tumours of the mediastinum—part II

et al. 2015

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Section: Tumours Of Uncertain Differentiationmentioning

confidence: 99%

“…Metastatic disease developed 16 months post-surgery; no further follow-up was provided. A second case of CCS was resected by VATS from the superior mediastinum of a 63-year-old male [ 194 ]. A tumour of 8.5 cm was removed and showed the typical histology for CCS with positive staining for HMB45 and S-100 protein.…”

Section: Tumours Of Uncertain Differentiationmentioning

confidence: 99%

Mesenchymal tumours of the mediastinum—part II

et al. 2015

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“…Of all published studies on CCSs, there are only 2 reported cases in the literature involving mediastinum [11, 12]. Morphologic appearances in both cases were similar to cutaneous melanoma.…”

Section: Discussionmentioning

confidence: 92%

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

et al. 2017

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BackgroundClear cell sarcoma (CCS) is a rare malignant soft-tissue neoplasm that displays melanocytic markers and exhibits striking histopathological features. The tumour has a predilection for the lower extremities and rarely presents in the mediastinum.Case presentationWe present a case of primary mediastinal CCS in a 57-year-old man. Computer tomography (CT) revealed a 12 × 12 × 7.5 cm mass in the anterior mediastinum. Microscopically, the tumour mainly consisted of epithelioid cells with oval vesicular nuclei and eosinophilic cytoplasm. Immunohistochemically, the tumour was positive for human melanoma black 45 (HMB-45) and vimentin but negative for S-100 and Melan-A. Fluorescence in situ hybridisation (FISH) showed a translocation involving the EWSR1 gene region.ConclusionThis report will illustrate that the mediastinum is a potential site for primary CCS and FISH plays an important role in making a conclusive diagnosis.

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“…The tumors frequently have an indolent presentation and can be clinically present many years before histologic diagnosis ( 47 , 48 ). A handful of these tumors have been reported as large masses arising in the mediastinum with the tumor cells exhibiting epithelioid features ( 13 , 49 , 50 ). Histologically, clear cell sarcomas are characterized by epithelioid to spindled cells with eosinophilic or clear cytoplasm.…”

Section: Other Sarcomas With Epithelioid Morphologymentioning

confidence: 99%

Sarcomas of the mediastinum with epithelioid morphology

Perry¹,

Montecalvo²,

Perry³

2021

Mediastinum

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While soft tissue sarcomas typically have a spindled or pleomorphic appearance, a subset of malignant soft tissue neoplasms can have a prominent epithelioid morphology. In complex anatomic sites such as the mediastinum, such tumors can often be mistaken for a carcinoma or mesothelioma. Frequent expression of cytokeratin staining can further confound the diagnostic process and familiarity with these entities can help prevent an erroneous diagnosis. Particular entities that have been reported to occur in the mediastinum with such features include dedifferentiated liposarcoma, pleomorphic liposarcoma, malignant peripheral nerve sheath tumor, synovial sarcoma, SMARCA4-deficient thoracic sarcoma, alveolar soft part sarcoma and clear cell sarcoma. Many of these tumors exhibit unique clinical, genetic, molecular or immunohistochemical features which allow for accurate characterization. For example, pleomorphic liposarcoma contains bizarre appearing lipoblasts and dedifferentiated liposarcoma exhibits MDM2 gene amplification that is typically confirmed by fluorescence in-situ hybridization. Malignant peripheral nerve sheath tumor will often arise in association with a nerve or neurofibroma. Synovial sarcoma consistently exhibits rearrangements involving the SS18 gene and SMARCA4-deficient thoracic sarcoma shows loss of SMARCA4 staining in the tumor cells. Alveolar soft part sarcoma demonstrates an ASPL-TFE3 fusion transcript. Clear cell sarcoma often shows an EWSR1-ATF1 fusion transcript. When encountering a sarcoma of the mediastinum with epithelioid features, familiarity with these and other characteristics can help insure a correct diagnosis.

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Primary Clear-Cell Sarcoma in the Mediastinum

Cited by 4 publications

References 7 publications

Mesenchymal tumours of the mediastinum—part II

Mesenchymal tumours of the mediastinum—part II

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

Sarcomas of the mediastinum with epithelioid morphology

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