睾丸旁促结缔组织增生性小圆细胞肿瘤1例报告及文献复习

目的:探讨睾丸旁促结缔组织增生性小圆细胞肿瘤的临床、病理特征、诊断及治疗。方法:回顾分析1例睾丸旁促结缔组织增生性小圆细胞肿瘤的诊治及随访资料,并复习相关文献。患者,男,27岁,因阴囊内无痛性肿块4个月就诊。体检于左侧阴囊内可扪及囊性肿块,同侧睾丸不能扪及。行左侧睾丸根治性切除术,术后辅以化疗。结果:术中见左侧睾丸旁多发结节状隆起肿块,质偏硬;术后病理检查肿瘤细胞呈巢状或梁索状结构并埋没在增生的纤维结缔组织中;免疫组化显示瘤细胞具有上皮源性、间质性和神经源性等多向分化的特点。术后已随访3年无瘤生存,预后良好。结论:促结缔组织增生性小圆细胞肿瘤有特异的临床病理特征,好发于年轻男性,手术切除肿瘤联合化疗是治疗的主要方法,睾丸旁促结缔组织增生性小圆细胞肿瘤比腹腔型预后相对好。

Desmoplastic Small Round-cell Tumor of the Paratesticular Region: A Case Report and Review of the Literature

OBJECTIVE: To investigate the clinical and pathological features of paratesticular desmoplastic small round cell tumor (DSRCT), and to improve the diagnosis and treatment of the disease. METHODS: One case of paratesticular DSRCT was studied retrospectively and a considerable amount of related literature from Medline and Chinese journals reviewed. The patient was a 27-year-old man presenting with a painless testicular mass in the left hemiscrotum. On physical examination, a cystic mass was palpable while the testis was not in the left hemiscrotum. RESULTS: During the operation the paratesticular area was found full of multiple nodular tumor masses of various sizes ranging from 0.5 cm to 1.5 cm in diamater. Pathological examination showed the characteristic histological pattern of nests of small undifferentiated cells embedded in a dense fibrous stroma. The tumor presented an immunohistochemical feature of epithelial, mesenchymal as well as neural multidirectional differentiation. Following testicular tumor orchiectomy, chemotherapy was performed with DDP, VP16, ifosfamide and EPI. Three years follow-up found no tumor recurrence. CONCLUSION: Desmoplastic small round cell tumor has a specific clinicopathologic stigmata, usually occurring in young males, for which surgical resection with chemotherapy is the treatment of choice. DSRCT located in the paratesticular region may have a better prognosis than its more frequently abdominal counterpart.