A Case of Clear-Cell Sarcoma in the Stomach.
10.3904/kjm.2015.89.4.465
- Author:
Jwa Hoon KIM
1
;
Beom Su KIM
;
Joon Seon SONG
;
Hyungwoo CHO
;
Jongkwan KIM
;
Joohee LEE
;
Min Hee RYU
Author Information
1. Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea. miniryu@amc.seoul.kr
- Publication Type:Case Report
- Keywords:
Sarcoma, Clear cell;
Stomach;
Paraneoplastic syndromes
- MeSH:
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
- From:Korean Journal of Medicine
2015;89(4):465-469
- CountryRepublic of Korea
- Language:Korean
-
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.
