PARATESTICULAR RHABDOMYOSARCOMA - RESULTS OF THERAPY IN 18 CASES
PARATESTICULAR RHABDOMYOSARCOMA - RESULTS OF THERAPY IN 18 CASES
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DOI：
10.1016/s0022-5347(17)39764-1
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发表时间：
1990-12-01
影响因子：
6.6
通讯作者：
RETIK, AB
中科院分区：
文献类型：
--
作者：
BLYTH, B;MANDELL, J;RETIK, AB
睾丸旁横纹肌肉瘤的治疗进展慢于睾丸生殖细胞肿瘤，主要是由于其发病率较低以及缺乏标准化的治疗方案。随着分期和治疗的逐步完善，预后已经消失。1960年至1988年，18名2-18岁的患者在美国马萨诸塞州波士顿的S儿童医院接受治疗。治疗包括腹股沟切除、分期评估和腹膜后淋巴结清扫，然后根据疾病的分期进行化疗和放射治疗。11例(61%)局限于阴囊，7例扩散至腹膜后淋巴结，其中3例有远处转移。组织学检查显示，除1种肺泡细胞类型外，其余均为胚胎特征。化疗方案：放线菌素D 2例，长春新碱、放线菌素D、环磷酰胺2例，长春新碱、放线菌素D、环磷酰胺脉冲剂10例，阿霉素加长春新碱、放线菌素D、环磷酰胺4例。17例患者无病，中位随访4年，死亡1例。无复发生存率和总生存率分别为和94%。我们得出结论，睾丸旁横纹肌肉瘤是横纹肌肉瘤的一个有利亚型。
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 removed. Between 1960 and 1988, 18 patients 2 to 18 years old were treated at the Children''s Hospital [Boston, Massachusetts, USA]. 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 rhabdomysarcoma represents a favorable subgroup of rhabdomyosarcoma.