Raney R B, Tefft M, Lawrence W, Ragab A H, Soule E H, Beltangady M, Gehan E A
Division of Pediatric Hematology-Oncology, University of Virginia Children's Medical Center, Charlottesville 22908.
Cancer. 1987 Nov 1;60(9):2337-43. doi: 10.1002/1097-0142(19871101)60:9<2337::aid-cncr2820600937>3.0.co;2-3.
This article reports on the diagnostic features, radiographic findings, staging, operative management, treatments, and treatment results for 95 children (mean age, 10 years) with paratesticular sarcoma; they were treated on the Intergroup Rhabdomyosarcoma Study (IRS) I and II protocols. Embryonal rhabdomyosarcoma (RMS) was the most common histologic subtype (97% of cases). Patients were randomly assigned to receive various therapeutic regimens according to IRS protocols. Among 81 patients (85%) with localized disease, most (57) were in Clinical Group I (localized, completely excised tumors), and 20 were in Group II (gross excision with tumor-involved, regional retroperitoneal lymph nodes or microscopic residual). Only four were in Group III (gross residual disease in the primary site or retroperitoneal lymph nodes). Three of them achieved a complete response (CR) after induction therapy and two then relapsed. The relapse-free survival (RFS) estimates at 3 years from diagnosis were 93% in Group I and 90% in Group II. Distant metastases were present at diagnosis in only 14 patients. Twelve of them achieved CR, but four subsequently relapsed; their 3-year RFS estimate was 67%. Overall, survival rates among the 95 children were excellent (89% at 3 years) compared to the entire IRS series (63%). Treatment with radical orchidectomy and chemotherapy was sufficient for Group I patients. Orchidectomy, chemotherapy, and radiation therapy were highly effective in Group II patients and in a large proportion of those with more advanced tumors. Recommendations for the diagnostic examinations and management of future patients with paratesticular sarcoma are given.
本文报告了95例睾丸旁肉瘤患儿（平均年龄10岁）的诊断特征、影像学表现、分期、手术管理、治疗方法及治疗结果；这些患儿按照横纹肌肉瘤协作组（IRS）的I期和II期方案接受治疗。胚胎型横纹肌肉瘤（RMS）是最常见的组织学亚型（占病例的97%）。患者根据IRS方案被随机分配接受不同的治疗方案。在81例（85%）局限性疾病患者中，大多数（57例）属于临床I组（局限性、肿瘤完全切除），20例属于II组（肿瘤累及区域腹膜后淋巴结或镜下残留的大体切除）。只有4例属于III组（原发部位或腹膜后淋巴结有大体残留病灶）。其中3例在诱导治疗后达到完全缓解（CR），但2例随后复发。诊断后3年的无复发生存率（RFS）估计在I组为93%，在II组为90%。仅14例患者在诊断时有远处转移。其中12例达到CR，但4例随后复发；他们3年的RFS估计为67%。总体而言，与整个IRS系列（63%）相比，这95例患儿的生存率极佳（3年时为89%）。对于I组患者，根治性睾丸切除术和化疗就足够了。睾丸切除术、化疗和放疗对II组患者以及大部分肿瘤更晚期的患者非常有效。文中给出了对未来睾丸旁肉瘤患者诊断检查和管理的建议。
