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Presented by Hind Nassar, M.D. and prepared by ChanJuan Shi, M.D., Ph.D.
Case 4: 71 year old woman with paraspinal thoracic mass.
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1. Question
Week 379: Case 4
71 year old woman with paraspinal thoracic massimages/nassar012609-4a.jpg
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images/nassar012609-4d.jpgCorrect
Answer: Clear cell sarcoma
Histology: see below
Discussion: Soft tissue clear cell sarcoma occurs usually in young adults. The main location is the extremities where it is associated with tendons and aponeuroses. Despite a slow course going for many years in some cases, the lesion is aggressive and can metastasize to lymph nodes, lungs, etc…It is difficult to differentiate clear cell sarcoma from melanoma based on morphology and immunohistochemistry. Both tumors are strongly positive for S100, HMB45, Mart-1 and MITF. Clinical history can be helpful but a definitive diagnosis of clear cell sarcoma could be made based on molecular studies by demonstrating the presence of a t (12, 22) involving the EWS1/ATF genes. Of note this translocation has been recently describe in another tumor, angiomatoid fibrous histiocytoma, a soft tissue tumor not related to clear cell sarcoma. On the other hand, clear cell sarcoma occurring in the GI tract has a different translocation than that observed in soft tissue clear cell sarcoma.
Incorrect
Answer: Clear cell sarcoma
Histology: see below
Discussion: Soft tissue clear cell sarcoma occurs usually in young adults. The main location is the extremities where it is associated with tendons and aponeuroses. Despite a slow course going for many years in some cases, the lesion is aggressive and can metastasize to lymph nodes, lungs, etc…It is difficult to differentiate clear cell sarcoma from melanoma based on morphology and immunohistochemistry. Both tumors are strongly positive for S100, HMB45, Mart-1 and MITF. Clinical history can be helpful but a definitive diagnosis of clear cell sarcoma could be made based on molecular studies by demonstrating the presence of a t (12, 22) involving the EWS1/ATF genes. Of note this translocation has been recently describe in another tumor, angiomatoid fibrous histiocytoma, a soft tissue tumor not related to clear cell sarcoma. On the other hand, clear cell sarcoma occurring in the GI tract has a different translocation than that observed in soft tissue clear cell sarcoma.
