Blyth B, Mandell J, Bauer S B, Colodny A H, Grier H E, Weinstein H J, Tarbell N J, Hendren W H, Retik A B
Department of Surgery, Children's Hospital, Dana Farber Cancer Institute, Boston, Massachusetts 02115.
J Urol. 1990 Dec;144(6):1450-3. doi: 10.1016/s0022-5347(17)39764-1.
The management of paratesticular rhabdomyosarcoma has evolved more slowly than that for testicular germ cell tumors, mainly due to its lower incidence and the lack of standardized treatment protocols. With gradual refinements in staging and therapy the prognosis has improved. Between 1960 and 1988, 18 patients 2 to 18 years old were treated at the Children's Hospital. Management consisted of inguinal orchiectomy, staging evaluation and retroperitoneal lymph node dissection, followed by chemotherapy and radiotherapy according to the stage of the disease. In 11 patients (61%) the disease was confined to the scrotum, while 7 presented with spread to the retroperitoneal lymph nodes, including 3 children with more distant metastases. Histological study revealed predominantly embryonal characteristics except for 1 alveolar cell type. Chemotherapy consisted of actinomycin D in 2 patients, vincristine, actinomycin D and cyclophosphamide in 2, pulse vincristine, actinomycin D and cyclophosphamide in 10, and doxorubicin plus vincristine, actinomycin D and cyclophosphamide in 4. A total of 17 patients remain free of disease (median followup 4 years) with 1 death. The actuarial survival without relapse and over-all survival rate are 89 and 94%, respectively. We conclude that paratesticular rhabdomyosarcoma represents a favorable subgroup of rhabdomyosarcoma.
睾丸旁横纹肌肉瘤的治疗进展比睾丸生殖细胞肿瘤更为缓慢，主要原因是其发病率较低且缺乏标准化治疗方案。随着分期和治疗方法的逐步完善，预后有所改善。1960年至1988年间，儿童医院共治疗了18例2至18岁的患者。治疗方法包括腹股沟睾丸切除术、分期评估和腹膜后淋巴结清扫术，然后根据疾病分期进行化疗和放疗。11例患者（61%）的疾病局限于阴囊，7例出现腹膜后淋巴结转移，其中3例儿童有更远处转移。组织学研究显示，除1例为肺泡细胞型外，主要为胚胎型特征。2例患者的化疗方案为放线菌素D，2例为长春新碱、放线菌素D和环磷酰胺，10例为脉冲长春新碱、放线菌素D和环磷酰胺，4例为阿霉素加长春新碱、放线菌素D和环磷酰胺。共有17例患者无病生存（中位随访4年），1例死亡。无复发生存率和总生存率分别为89%和94%。我们得出结论，睾丸旁横纹肌肉瘤是横纹肌肉瘤中预后较好的一个亚组。
