Clear cell sarcoma like tumor of gastrointestinal tract: Experience of three cases and review of literature

Abstract

Clear cell sarcoma-like tumor of the gastrointestinal tract (CCSGT) is a rare, aggressive tumor with many histological mimickers. Herein, we have documented our experience of three cases of CCSGT and reviewed the literature. The index cases were identified in male patients in their twenties, one in jejunum and two in the distal colon. Histomorphological examination revealed the characteristic heterogeneous histomorphology with patchy immunohistochemical positivity with S100 protein and negative melanocytic markers. The fluorescence in-situ hybridization test showed translocation of the EWSR1 (22q12) gene in >80% tumor cells. While one of our patients died after 2 years with lung metastasis, the other two patients are still alive on 1.5 years and 3 months follow up, respectively. CCSGT is a rare malignant tumor of the gastrointestinal tract. Although characteristic morphology, use of a judicial panel of immunohistochemical stains, and translocation study for EWSR1 gene can establish the diagnosis, experience in adjuvant therapy is still limited.