scholarly journals Clear Cell Sarcoma (Malignant Melanoma of Soft Part): A Rare Soft Tissue Sarcoma

2016 ◽  
Vol 10 (1) ◽  
Author(s):  
Muhammad Abdullah

Clear cell sarcoma is a rare malignant neoplasm usually presenting as soft tissue mass in foot of young adult. Ultrastructural studies reveal neuroectodermal origin with melanocytic differentiation. Thus it is considered as malignant melanoma of soft parts. Though the prognosis is apparently better as compared to the conventional melanoma, local recurrences and metastases are frequently seen. Large tumor size and extent of necrosis are factors for poor prognosis. A case of clear cell sarcoma with classical presentation is reported.

2010 ◽  
Vol 63 (5) ◽  
pp. 416-423 ◽  
Author(s):  
Kemal Kosemehmetoglu ◽  
Andrew L Folpe

Clear cell sarcoma (CCS) is a rare, distinctive soft tissue neoplasm, typically occurring in the distal extremities of young adult patients. Although CCS shows melanocytic differentiation, it is now clear that it is clinicopathologically and genetically distinct from conventional malignant melanoma. The ‘osteoclast-rich tumour of the gastrointestinal tract with features resembling clear cell sarcoma of soft parts’ is an extraordinarily rare gastrointestinal neoplasm that shares some features of CCS, but differs from it in other ways. The historical, histopathological, ultrastructural, immunohistochemical and genetic aspects of these two tumours are reviewed in this article.


2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S29-S30
Author(s):  
C M Tucker ◽  
J Liu ◽  
S M Gargano ◽  
C E Fundakowski

Abstract Casestudy: Clear cell sarcoma of soft tissue (CCSST) is a rare translocation-associated sarcoma which uncommonly presents in the head and neck region. Because the tumor expresses markers of melanocytic differentiation, it can be difficult to distinguish from malignant melanoma and epithelioid malignant peripheral nerve sheath tumor (MPNST), necessitating clinical correlation and ancillary testing specifically fluorescence in situ hybridization (FISH) for EWSR1 rearrangement. Here we report a case of CCSST involving the premaxillary soft tissue. A 61-year-old male without significant past medical history presented with a right premaxillary subcutaneous mass which was slowly enlarging over the course of three years. During surgical resection of the mass, the infraorbital nerve was noted to course into the tumor. Histologic sections revealed a 2.5 cm mass composed of a nodular proliferation of epithelioid tumor cells with pale eosinophilic to vacuolated cytoplasm, large vesicular nuclei with prominent nucleoli, areas of punctate necrosis, and a mitotic rate of 10 mitoses per 10 high power fields. Scattered multinucleated tumor giant cells were also present. No pigment was identified. By immunohistochemistry, the tumor cells were diffusely positive for SOX-10 and S-100 and negative for HMB45, MART1, MITF, inhibin, calretinin, p63 and smooth muscle actin. FISH was positive for EWSR1 rearrangement. Altogether, the morphologic, immunohistochemical and cytogenetic findings supported the diagnosis of CCSST. Because the surgical margins were negative for malignancy, the patient did not require additional surgery but will be receiving adjuvant radiation therapy. Although rare in the head and neck, CCSST should be considered in the differential diagnosis when dealing with an epithelioid soft tissue tumor that expresses any melanocytic markers, and a definitive distinction from malignant melanoma and epithelioid MPNST can be achieved by utilizing FISH for EWSR1 rearrangement.


2009 ◽  
Vol 87 (6) ◽  
pp. 1927-1928 ◽  
Author(s):  
Gaetano Rocco ◽  
Anna Rosaria de Chiara ◽  
Flavio Fazioli ◽  
Francesco Scognamiglio ◽  
Antonello La Rocca ◽  
...  

2003 ◽  
Vol 30 (7) ◽  
pp. 550-555 ◽  
Author(s):  
Takuya Seike ◽  
Kazuya Matsumoto ◽  
Hideki Nakanishi ◽  
Ichiro Hashimoto ◽  
Yoshiaki Kubo ◽  
...  

2006 ◽  
Vol 21 (6) ◽  
pp. 1-4 ◽  
Author(s):  
Ahmet Şengöz ◽  
Erol Taşdemiroğlu ◽  
Halit Togay

✓The authors present a case of clear cell sarcoma (CCS) in which the tumor originated in the S-1 nerve root and had been previously diagnosed as psammomatous melanotic schwannoma (PMS). This is the third case of a spinal nerve root origin for CCS reported in the English-language literature. The similar histogenesis of CCS and malignant melanoma supports the hypothesis that biological agents or immunotherapy are potentially important areas of investigation. The patient underwent S1–3 laminectomy and gross-total resection of the mass lesion. The border of the resection was extended 1 cm distal to the tumor margin. The postoperative period was uneventful. The new histopathological diagnosis was CCS (malignant melanoma of soft tissue). Despite total resection, the patient returned with disseminated disease at the 18-month follow-up visit. His follow-up magnetic resonance image of the lumbar spine revealed sacral L5–S3 involvement of the vertebral bodies along with disseminated cauda equina seeding. A CCS originating from peripheral nerves is quite rare. The histopathological and immunohistochemical appearance of CCSs resembles those of PMSs. Surgery should be the first choice of treatment.


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