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결합조직형성 소원형세포암은 젊은 남자의 복강에 발 생하는 드문 종양으로 비교적 최근에 독립된 질환으로 분류되었으며, 아직 표준화된 치료법이 없는 실정이다.


Desmoplastic small round cell tumor (DSRCT) is a rare neoplasm that develops in the abdominal cavity in young people. Microscopically, nests of small undifferentiated cells within a desmoplastic stroma are observed and immunohistochemical studies show polyphenotypic differentiation with expression of epithelial, neural and muscle markers. A specific chromosomal translocation, t (11;22)(p13;q12) has been documented in DSRCT and is increasingly used to confirm the diagnosis. We had experienced a 59-year-old female with right upper quardrant pain for 1 month. On abdominal computerized tomography, multiple demarcated intrahepatic mass lesions were found, but other organs metastasis or abnormal lymph node enlargement and fluid collection were not found. Fine needle aspiration biopsy of hepatic mass was performed and DSRCT was diagnosed by H&E and immunohistochemical staining. She was treated with cyclophosphamide, etoposide, doxorubicin and cisplatin. After three cycles of chemotherapy, computerized tomography demonstrated distinct shrinkage of tumor. At present, she continues in the state of partial remission for 15months after chemotherapy was started.(Korean J Med 65:610-614, 2003)


Desmoplastic small round cell tumor (DSRCT) is a rare neoplasm that develops in the abdominal cavity in young people. Microscopically, nests of small undifferentiated cells within a desmoplastic stroma are observed and immunohistochemical studies show polyphenotypic differentiation with expression of epithelial, neural and muscle markers. A specific chromosomal translocation, t (11;22)(p13;q12) has been documented in DSRCT and is increasingly used to confirm the diagnosis. We had experienced a 59-year-old female with right upper quardrant pain for 1 month. On abdominal computerized tomography, multiple demarcated intrahepatic mass lesions were found, but other organs metastasis or abnormal lymph node enlargement and fluid collection were not found. Fine needle aspiration biopsy of hepatic mass was performed and DSRCT was diagnosed by H&E and immunohistochemical staining. She was treated with cyclophosphamide, etoposide, doxorubicin and cisplatin. After three cycles of chemotherapy, computerized tomography demonstrated distinct shrinkage of tumor. At present, she continues in the state of partial remission for 15months after chemotherapy was started.(Korean J Med 65:610-614, 2003)