Treatment of @entity1 with nonmetastatic @entity980 : results of the Malignant Mesenchymal Tumors studies ( XXXX 84 and @entity569 89) of the International Society of Pediatric Oncology.

@entity1 @entity5 @entity569 @entity4316 @entity1891 @entity1260 @entity980 @entity1349
PURPOSE: To report the results of the Malignant Mesenchymal Tumors studies ( @entity569 84 and 89) of the International Society of Pediatric Oncology (SIOP) in males with nonmetastatic @entity980 . @entity1 AND METHODS: From 1984 to 1994, 96 males were treated in SIOP protocols. Radical inguinal orchidectomy was recommended, but initial retroperitoneal lymph node dissection was not performed. Disease was staged according to the SIOP @entity5 -node-metastasis staging system. Treatment was stratified by stage. In the @entity569 89 study, males with completely resected @entity5 at diagnosis received less chemotherapy ( @entity1891 and @entity4316 ) than @entity1 in the @entity569 84 study ( @entity1260 , @entity1891 , and @entity4316 ). RESULTS: Median age at diagnosis was 65 months. Thirty-one @entity5 were larger than 5 cm, and 13 males were older than 10 years with a @entity5 larger than 5 cm. At a median follow-up of 7 years, 87 @entity1 were alive; 79 were in first complete remission and eight were in second complete remission. Relapse occurred in 16 @entity1 (17%). At 5 years, the @entity1349 ( @entity1349 ) rate was 92%, with an event-free survival (EFS) rate of 82%. @entity1349 and EFS were significantly worse for males with @entity5 greater than 5 cm and for males older than 10 years at diagnosis. CONCLUSION: Males with paratesticular RMS have an excellent prognosis except for a selected group of @entity1 older than 10 years or with @entity5 greater than 5 cm. Intensified chemotherapy incorporating alkylating agents for this subgroup may be preferred to the use of systematic lymphadenectomy to improve survival while minimizing the burden of therapy.
