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[目的]探讨腹部促结缔组织增生性小圆细胞肿瘤(DSRCT)的组织学形态、免疫学表型及诊断和鉴别诊断的要点。[方法]回顾性复习4例DSRCT的临床资料、组织学形态和免疫学表型。[结果]临床上DSRCT多表现为腹胀、腹痛、腹部包块。组织学上DSRCT由成团巢的小圆细胞和丰富的结缔组织间质组成。免疫学表型DSRCT显示多向分化,表达上皮性、神经源性和肌源性等标记,Vimentim和Desmin呈特征性的核旁点状染色。电镜下特征结构为核旁漩涡状排列的中间丝呈小球聚集。[结论]DSRCT主要发生于腹腔或盆腔,肿瘤由具有多向分化的高度恶性的小圆细胞和丰富的结缔组织间质组成,免疫组化Vimentim和Desmin核旁点状染色在诊断和鉴别诊断中具有重要价值。
[Objective] To investigate the histological and immunological phenotypes of the connective tissue hyperplastic small round cell tumor (DSRCT) in the abdomen and the main points of diagnosis and differential diagnosis. [Methods] Retrospective review of 4 cases of DSRCT clinical data, histological morphology and immunological phenotype. [Results] DSRCT clinically manifested as abdominal distension, abdominal pain and abdominal mass. Histologically, DSRCT consists of parietal cells and abundant interstitial connective tissue. Immunological phenotype DSRCT showed multi-directional differentiation, expression of epithelial, neurogenic and myogenic and other markers, Vimentim and Desmin characteristic nucleus punctate staining. Electron microscopy of the characteristic structure of the paraxial arrangement of intermediate filaments gathered beads. [Conclusion] DSRCT mainly occurs in abdominal cavity or pelvic cavity. The tumor consists of highly malignant small round cells with multi-directional differentiation and abundant connective tissue interstitial. Dot-like staining of immunohistochemical Vimentim and Desmin nuclei in the diagnosis and differential diagnosis Has important value.