Paratesticular sarcoma in childhood and adolescence. A report from the Intergroup Rhabdomyosarcoma Studies I and II, 1973-1983.

Abstract

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.

• Authors: R B Raney Jr, M Tefft, W Lawrence Jr, A H Ragab, E H Soule, M Beltangady, E A Gehan
• Journal: Cancer (Cancer) Vol. 60 Issue 9 Pg. 2337-43 (Nov 01 1987) ISSN: 0008-543X [Print] United States
• PMID: 3440240 (Publication Type: Clinical Trial, Journal Article, Randomized Controlled Trial, Research Support, U.S. Gov't, P.H.S.)

Topics

• Adolescent
• Child
• Child, Preschool
• Combined Modality Therapy
• Humans
• Lymph Node Excision
• Lymphatic Metastasis
• Male
• Neoplasm Recurrence, Local
• Neoplasm Staging
• Orchiectomy
• Prognosis
• Rhabdomyosarcoma (diagnosis, secondary, therapy)
• Sarcoma (diagnosis, secondary, therapy)
• Testicular Neoplasms (diagnosis, therapy)