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 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.
Raney
R B
RB
Division of Pediatric Hematology-Oncology, University of Virginia Children's Medical Center, Charlottesville 22908.
Tefft
M
M
Lawrence
W
W
Ragab
A H
AH
Soule
E H
EH
Beltangady
M
M
Gehan
E A
EA
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D006801:Humans; D008197:Lymph Node Excision; D008207:Lymphatic Metastasis; D008297:Male; D009364:Neoplasm Recurrence, Local; D009367:Neoplasm Staging; D009919:Orchiectomy; D011379:Prognosis; D012208:Rhabdomyosarcoma / Q000175:diagnosis / Q000556:secondary / Q000628:therapy*; D012509:Sarcoma / Q000175:diagnosis / Q000556:secondary / Q000628:therapy*; D013736:Testicular Neoplasms / Q000175:diagnosis / Q000628:therapy*