Clear-
cell sarcoma (CCS) is a rare
soft tissue sarcoma that usually develops in the
lower extremities of
young adults. CCS of the
gastrointestinal tract is extremely rare. We
report here the first case of CCS of the
stomach in
Korea. A 28-year-old
female developed a large
tumor of the
stomach that was initially considered
Ewing sarcoma, based on the results of a
needle biopsy. She had
paraneoplastic syndrome;
fever,
anemia, and
hyperglycemia. Follow-up abdominal computed
tomography after four-cycles of
chemotherapy with
vincristine-
doxorubicin-
cyclophosphamide or
ifosfamide-
etoposide showed no
tumor shrinkage and revealed a
fistula between the
tumor and the gastric lumen. The infected
tumor prompted debulking
surgery, which resulted in improvement of her symptoms. The pathologic findings were consistent with CCS, and fluorescence in situ hybridization for EWS
gene rearrangement was positive, confirming the
diagnosis. This case highlights the importance of clinical suspicion for CCS in
chemotherapy-refractory
Ewing sarcoma.