原发性附睾横纹肌肉瘤一例报告

目的 探讨原发性附睾横纹肌肉瘤的临床特征及诊治进展. 方法总结1例原发性附睾横纹肌肉瘤患者资料并复习文献.患者16岁,发现右阴囊肿物4个月入院.体检:右侧阴囊内4.5 cm×3.5 cm×3.0 cm卵圆形实性肿物,光滑、质硬、无压痛,透光试验阴性,腹股沟未及肿大淋巴结.术前诊断:右附睾炎性结节. 结果 骶管麻醉下行右附睾切除术.病理检查示肿瘤细胞呈小圆形,核小而深染.免疫组化染色:肌红蛋白(++++),肌动蛋白(+++),结蛋白(+++),平滑肌肌动蛋白(一).电镜下见瘤细胞胞质内较多平行排列的细肌丝,未见肌节样结构.病理诊断:右附睾胚胎性横纹肌肉瘤.患者家属拒绝进一步治疗.术后2个月右阴囊内再次发现肿块,生长迅速并疼痛,考虑附睾肿瘤复发.行右睾丸根治性切除术.冰冻切片示切缘无肿瘤细胞,病理诊断:右阴囊横纹肌肉瘤(复发).给予异环磷酰胺、长春新碱、依托泊苷联合化疗.随访1年,未见复发. 结论 附睾原发性横纹肌肉瘤罕见,进展快,临床表现无特异性,诊断主要依赖病理检查.根治性睾丸切除术加辅助化疗和放疗是主要治疗方法,预后好.

Primary epldidymul rhabdomyosarcoma; one ease report

Objective To discuss the clinical features of primary epididymal rhabdomyosarcoma and review the diagnostic and therapeutic advances. Methods One 16-yesr-old male presented with right scro-turn swelling mass for 4 months. Physical examination revealed an ovoid and solid mass of 4.5×3.5×3.0 cn in right scrotum. The mass was hard and its surface was smooth without tenderness. Transillumination test was negative. There was no palpable inguinal lymph node. Pre-operative diagnosis was inflammatory nodule of right epididymis. Results Right epididymectomy was performed under caudal anesthesia une-ventfully. The pathological study showed that the neoplasm was composed of small round shape ceils with small and anachromasis nucleus. The immunohistochemical study showed that these cells were positive for myoglobin, aetin and desmin, but negative for smooth muscle actin. Electron microscopy showed that paral-leling thin filaments were found with no asrcomere-like structure in the intracytoplasm. The pathologic diag-nosis was right epydidymal rhabdomyosarcoma. The patient refused further therapy. A mass was found with rapid growth accompanied with pain in the right scrotum 2 months after the first operation and was clinically diagnosed as tumor recurrence. This patient had then undergone right radical orchiectomy. Frozen section analysis revealed negative surgical margin. The pathologic diagnosis was embryonal rhabdomyosarcoma re-currence. Patient underwent subsequent chemotherapy with ifosfamide, etoposide and vincristine. After one year follow-up, he was generally well without evidence of recurrence. Conculsions Primary embryonal rhabdomyosarcoma is a rare tumor with aggressive growth patterns. The diagnosis depends on the pathologi-cal examination because the clinical presentation is not specific. Combination therapy with radical orchiecto-my, chemotherapy and radiotherapy provides patients an excellent long-term prognosis.