Zhonghua wai ke za zhi [Chinese journal of surgery] · 2002
[Desmoplastic small round cell tumor: report of 2 cases].
Клинический случайАннотация на английскомСтатьяНазвание и аннотация: Перевод пока не подготовлен
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Hu Zili, Leng Zhi, Zhang Weili, Qiao Tianyu, Yang Guangyong, Xu Guangyong, Liu Chuan
Аннотация
OBJECTIVE: To recognize the characteristics of desmoplastic small round cell tumor (DSRCT) and improve the standard of diagnosis.
METHODS: We retrospectively reviewed the clinical data on the treatment of 2 patients with DSRCT in terms of their conditions, tissue sources, pathologic characteristics, immunohistochemical methods, clinical manifestation, diagnosis, treatment and prognosis.
RESULTS: Clinical manifestations were complicated. The 2 patients were mis diagnosed before operation. Their tumors consisted of irregular nests of small and round cells, with nuclear hyperchromatism and scant cytoplasm embedded in a plenty of fibrous connective tissues. The edge of the nest was clear, with different sizes and shapes. Immunohistochemically, the 2 patients were positive for CK or EMA, NSE, des and vim of the epithelium, nerve, muscle and interstitial. They died 9 months after operation.
CONCLUSIONS: The tumor may occur in the abdomen, pelvic cavity and other sites, with different clinical manifestations. Routine examination should be replaced by immunohistochemical test for correct diagnosis of the tumor. Prognosis of most patients is not good.
METHODS: We retrospectively reviewed the clinical data on the treatment of 2 patients with DSRCT in terms of their conditions, tissue sources, pathologic characteristics, immunohistochemical methods, clinical manifestation, diagnosis, treatment and prognosis.
RESULTS: Clinical manifestations were complicated. The 2 patients were mis diagnosed before operation. Their tumors consisted of irregular nests of small and round cells, with nuclear hyperchromatism and scant cytoplasm embedded in a plenty of fibrous connective tissues. The edge of the nest was clear, with different sizes and shapes. Immunohistochemically, the 2 patients were positive for CK or EMA, NSE, des and vim of the epithelium, nerve, muscle and interstitial. They died 9 months after operation.
CONCLUSIONS: The tumor may occur in the abdomen, pelvic cavity and other sites, with different clinical manifestations. Routine examination should be replaced by immunohistochemical test for correct diagnosis of the tumor. Prognosis of most patients is not good.