促结缔组织增生性小圆细胞瘤的临床病理特征

Clinical and pathological features of desmoplastic small round cell tumor

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。

Purpose: To study the clinical and pathological features of desmoplastic small round cell tumor. Methods: Clinicopathologic data of 4 cases of desmoplastic small round cell tumor were presented with review of the literature, immunohistochemical staining ( EnVision method) for CK, EMA, Desmin, Vim, NSE, Actin, etc. were performed. Results: All cases were young males. The clinical presentation was intra-abdominal in three patients and para-testicular in one patients. The connective tissue was significantly proliferated around the tumor cell nests. Immunohistochemical staining revealed the tumor exepresses CK, EMA, Desmin, Vim and NSE. The two patients without chemotherapy died 10 months and 15 months respectively after the diagnosis. The tumors of the other two patients with chemotherapy had no progress after two cycles. Conclusions: Desmoplastic small round cell tumor is a malignant sarcoma occurring usually in young males. Desmin perhaps is the most useful diagnostic marker with a perinuclear dot-like pattern. Surgical resection with chemotherapy and radiotherapy might be the proper approach to this rare malignancy.