Clinical, pathological, and genetic profile of clear cell sarcoma-like tumour of jejunum: report of a rare aggressive tumour of small bowel.

Clear cell sarcoma-like tumour of the gastrointestinal tract (CCSLGT) is a rare entity which has been recently described as late as 2003. Only around 70 cases have been reported in the English literature till date. CCSLGT is mostly seen in young adults in the late 20 s and early 30 s. CCSLGT are aggressive tumours. They are similar to the clear cell sarcoma of the soft tissue but lack melanocytic differentiation, retain neuroendocrine differentiation, and have osteoclastic giant cells. EWSR1-CREB1 fusion is characteristic of these tumours. Complete surgical excision is the best treatment option available. They have a high recurrence rate and poor prognosis. Currently, effective chemotherapy or a targeted agent is not available for the management of these tumours. Here, we describe a case of clear cell sarcoma-like tumour of jejunum encountered by us in a young man. The immunohistochemical and genetic profiling of these tumours are also discussed.