原发性附睾胚胎型横纹肌肉瘤1例并文献复习

Study on one case of primary epididymal rhabdomyosarcoma

目的:探讨附睾横纹肌肉瘤的临床表现、病理和诊治方法。方法:回顾分析1例原发性附睾横纹肌肉瘤患者资料并复习相关文献。患者23岁,因偶然发现左阴囊肿物3周入院。查体左附睾尾部约3cm×3cm×1.5cm大小肿物,质硬,表面不规则,无压痛。MRI左附睾肿瘤可能性大。术中冰冻活检明确肿瘤后予以行左侧睾丸附睾根治性切除。10d后行腹腔镜腹膜后淋巴结清扫术,术后予以化疗。结果:左侧睾丸附睾根治性切除术后病理:镜下见肿瘤细胞呈小圆形,核异型性明显,部分胞浆可见明显横纹。免疫组化结果:VIM(+)、MyoD1(+)、Myogenin(+)、Ki67(30%)、Desmin(+),诊断:左附睾横纹肌肉瘤。腹膜后淋巴结清扫未见淋巴转移。现予以化疗,未见复发及转移。结论:附睾胚胎型横纹肌肉瘤极罕见,临床表现不具特异性,确诊主要靠病理,预后较差,特别是10岁以上患者,是一种恶性程度高的肿瘤。

Objectives： To explore the clinical manifestation,pathology and treatment methods of epididymis leiomyosarcoma. Methods： A 23-year-old male patient presented with left scrotum swelling mass for 3 weeks was studied. The physical examination revealed an ovoid and solid mass of 3 × 3 × 1. 5cm in left tail of epididymis.The mass was hard and with irregular surface. It was very possible a left epididymis tumor with MRI. Intraoperative frozen biopsy was performed to remove the left testis and epididymis and laparoscopic retroperitoneal lymph node dissection after10 days. Chemotherapy was performed after operation. Results： Radical resection of the left testis and epididymis was performed. The pathological study showed that the neoplasm was composed of small round shape cells with small and anaehromasis nucleus. Stripes were found in part of the cytoplasm. The immunohis-tochemical study showed that these cells were positive for VIM,Myo D1,Myogenin,Ki67 and Desmin. The pathologic diagnosis was embryonal rhabdomyosareoma. Retroperitoneal lymph node dissection showed no tumor metastasis. Chemotherapy was performed and no metastasis was found. Conclusion： Primary embryonal rhabdomyossrcoma is a rare tumor,whose diagnosis depends highly on the pathological examination since the clinical presentation is not specific. It＇s a highly malignant tumor with poor prognosis especially for those over 10 years old.