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Paratesticular sarcoma in childhood and adolescence: A report from the intergroup rhabdomyosarcoma studies I and II, 1973–1983

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 micro‐scopic 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.