Korean J Med.  2015 Oct;89(4):465-469. 10.3904/kjm.2015.89.4.465.

A Case of Clear-Cell Sarcoma in the Stomach

Affiliations
  • 1Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea. miniryu@amc.seoul.kr
  • 2Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
  • 3Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
  • 4Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Abstract

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.

Keyword

Sarcoma, Clear cell; Stomach; Paraneoplastic syndromes

MeSH Terms

Adult
Anemia
Biopsy, Needle
Diagnosis
Drug Therapy
Female
Fever
Fistula
Fluorescence
Follow-Up Studies
Gastrointestinal Tract
Gene Rearrangement
Humans
Hyperglycemia
In Situ Hybridization
Korea
Lower Extremity
Paraneoplastic Syndromes
Sarcoma*
Sarcoma, Clear Cell
Sarcoma, Ewing
Stomach*
Young Adult
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