Primary chemotherapy in @entity980 and other XXXX of the orbit: results of the International Society of Pediatric Oncology MMT 84 Study.

@entity1 @entity8 @entity3480 @entity361 @entity5 @entity569 @entity4316 @entity1260 @entity980 @entity1370
PURPOSE: The @entity569 84 multicentric prospective trial of the International Society of Pediatric Oncology (SIOP) was designed to (1) test the effectiveness of @entity1260 3 g/m2 on days 1 and 2, vincristine 1.5 mg/m2 on days 1 and 14, and @entity4316 0.9 mg/m2 on days 1 and 2 ( @entity3480 ) repeated every 21 days; and (2) reduce late effects of treatment by reserving radiation therapy to the primary site for @entity1 not achieving a complete response (CR) to primary chemotherapy. MATERIALS AND METHODS: Between 1984 and 1989, the MMT 84 study registered 34 @entity1 with nonmetastatic @entity980 ( @entity980 ) and other @entity361 ( @entity361 ) of the orbit in this trial. RESULTS: The 4-year event-free survival rate is 62% +/- 9% (SD) and the 4-year survival rate 86% +/- 7% (SD). A total of 11 local recurrences occurred, 10 among 22 @entity1 treated without initial radiation. Salvage of local failure was achieved in nine of 11 @entity1 with the use of radiation and additional chemotherapy, but three later developed distant metastases and two have died. One isolated regional lymph node failure has occurred, while no @entity1 relapsed with isolated distant metastases. Six of 12 @entity1 who failed are alive with no evidence of disease from 16 to 50 months after relapse. The treatment was well tolerated in all @entity1 , except for one with @entity8 and one who died of @entity1370 . Twelve @entity1 remain in first remission without the use of radiation to the @entity5 from 27 to 84 months. CONCLUSION: Despite a higher incidence of local recurrence when treated by primary chemotherapy, early survival rates were not compromised and a significant number of @entity1 avoided the late effects of radiation. However, longer follow-up is required to assess the ultimate outcome of @entity1 treated in this manner.
