학술논문
위에 발생한 투명세포육종
A Case of Clear-Cell Sarcoma in the Stomach
A Case of Clear-Cell Sarcoma in the Stomach
Document Type
Article
Author
김좌훈 / Jwa Hoon Kim; 김범수 / Beom Su Kim; 송준선 / Joon Seon Song; 조형우 / Hyungwoo Cho; 김종관 / Jongkwan Kim; 이주희 / Joohee Lee; 류민희 / Min Hee Ryu
Source
대한내과학회지 (Korean J Med) / The Korean Journal of Medicine (Korean J Med). Oct 31, 2015 89(4):465
Subject
Language
Korean
ISSN
1738-9364
Abstract
위에 발생한 투명세포육종은 매우 드물며 국내에서 첫 증례를 보고하는 바이다. 투명세포육종의 진단은 어렵지만 충분한 조직 검체를 이용하여 면역조직화학 검사 및 분자학적 검사를 하면 진단할 수 있고, 특히 EWS 유전자 전좌를 확인하는 것이 중요하다. 문헌 보고에 따르면 투명세포육종의 치료로서 항암화학요법의 효과는 미미하였고 아직까지 항암화학요법에 대해 일치된 의견이 없어 현재까지 수술적 절제가 권장된다. 본 증례에서 초기 유잉 육종 진단하에 항암화학요법을 적용하였는데 치료 경과가 유잉 육종과 일치하지 않아투명세포육종을 의심하에 수술적 절제로 진단 및 치료를 하였다. 수술적 절제 이후에 부종양 증후군이 호전되는 것을 확인하였고 재발 없이 추적관찰 중이다.
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. (Korean J Med 2015;89:465-469)
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. (Korean J Med 2015;89:465-469)