Paratesticular rhabdomyosarcoma: a comparison of two Italian studies

Objective. To evaluate the treatment strategies adopted for children with paratesticular rhabdomyosarcoma (PT-RMS) in two consecutive protocols coordinated by the Italian Cooperative Group on Childhood Soft Tissue Sarcoma. Methods. We considered the clinical data and results of 34 patients with localized PT-RMS treated in Centers affiliated with the Associazione Italiana di Ematologia e Oncologia Pediatrica (AIEOP, Italian Association of Hematology and Pediatric Oncology) and included in the RMS79 (January 1979-December 1986) and RMS88 (January 1988-December 1995) protocols. A multidisciplinary approach that included surgery, chemotherapy and radiotherapy was adopted according to disease extension, histology subtype and result of initial surgery. However treatment intensity was reduced in the RMS88 study, by excluding both lymph node biopsy at diagnosis for staging purpose and alkylating agents from chemotherapy. Results. Ten year overall survival was 100% and 95% in the RMS79 and RMS88 protocol respectively, with a non statistically significant difference. Conclusions. A short treatment with vincristine and actinomycin is effective in patients with localized PT-RMS completely resected at diagnosis. Systematic exploration of retroperitoneal lymph nodes could be limited to patients with enlarged lymph nodes at CT scan