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目的:分析促结缔组织增生性小圆细胞瘤的临床表现,病理形态学及免疫表型特征。方法:分析4例促结缔组织增生性小圆细胞瘤患者的临床资料,对其标本进行大体和镜下观察,对石蜡切片行CK,EMA,Desmin,Vim,NSE,Actin等免疫组织化学EnVisionTM法染色。结果:4例患者均为男性,平均年龄22岁。3例发生于腹腔,1例发生于睾丸。组织学上由大小不一的细胞巢组成,巢周围结缔组织显著增生,瘤细胞表达CK,EMA,Desmin,Vim及NSE。2例未行化疗,分别存活10月和15月,2例行全身化疗两周期后病灶稳定无进展。结论:促结缔组织增生性小圆细胞瘤是一种好发于男性青少年的高度恶性小圆细胞性肉瘤。Desmin核旁点状染色具有诊断价值。手术切除肿瘤联合化放疗是治疗的主要方法。
OBJECTIVE: To analyze the clinical manifestations, pathomorphology and immunophenotypic features of connective tissue proliferative small round cell tumor. Methods: The clinical data of 4 patients with connective tissue proliferative small cell tumor were analyzed. The specimens were observed under microscope and in general. The immunohistochemical EnVisionTM method of CK, EMA, Desmin, Vim, NSE and Actin dyeing. Results: All four patients were male, with an average age of 22 years. Three cases occurred in the abdominal cavity and one in the testis. Histologically, cells of different sizes were formed, and connective tissue around the nest was significantly proliferated. The tumor cells expressed CK, EMA, Desmin, Vim and NSE. Two patients did not receive chemotherapy and survived in October and 15 months respectively. After 2 cycles of 2 cycles of systemic chemotherapy, the lesions were stable and without progression. Conclusions: The connective tissue proliferative small round cell tumor is a highly malignant small round cell sarcoma that occurs in male adolescents. Desmin nuclear punctate staining has diagnostic value. Surgical resection of tumor combined with radiotherapy is the main method of treatment.