Desmoplastic Small Round Cell Tumor-Reprot of 3 Cases And Review of the Literature

促纖維化小圓細胞腫瘤(Desmoplastic small round cell tumor，DSRCT)是一個有特殊組織學和免疫染色特徵的罕見惡性腫瘤。在組織學上可見纖維化的間質中有許多成團的未分化之小圓腫瘤細胞。第一個病例最早是由Gerald和Rossai於1989 年發表，通常好發於年青人的腹膜而且男性病患比女性多。雖然其組織學和免疫染色特徵非常明確，但若病理醫師對此病缺乏認識，則很容易誤診。在此提出本院的三個病例並加以討論。

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Desmoplastic small round cell tumor (DSRCT) is a rare neoplasm with distinctive histological features, characterized by extensive stromal tissue around islands of small and undifferentiated cells revealing the desmoplastic appearance. It was first described by Gerald and Rosai. It mainly the peritoneum and is found predominently in adolescents and young adults, and it is much more common in males than in females. The histological and immunhistochemical features are very distinct but may be misdiagnosed as other undifferentiated neoplasm if the pathologist has no idea of this entitiy. We report the experience of three cases here. The first case was diagnosed as malignant neuroendocrine neoplasm orginally and revised to DSRCT later. Case 2 had been diagnosed as Atypical carcinoid and received surgery and chemotherapy. It was revised as DSRCT 5 years later by an other pathologist. Case 3 carried the characteristic feature whereby diagnosis could be made even in frozen section.

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desmoplastic small round cell tumor

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Desmoplastic Small Round Cell Tumor-Reprot of 3 Cases And Review of the Literature

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