Long term survival in five rare cases with multiple primary neuroblastomas.

Purpose: Multiple primary neuroblastomas are a very uncommon disorder in infancy. The tumors may arise simultaneously in different parts of the sympathetic nervous system. The purpose of this paper was to present 5 new cases with multifocal neuroblastomas. This is a representative study based on the largest clinical material in Bulgaria.

Patients And Methods: The records of children treated for neuroblastoma during a 26 year-period (1979-2005) were reviewed and analysed retrospectively.

Results: Out of 193 children treated for neuroblastoma and followed in our department, 5 (2.6%) had multifocal lesions. All were boys aged from 2 months to 4 years at the time of diagnosis, and all were treated successfully with radical surgery. Four children were also administered chemotherapy with vincristine, cyclophosphamide and epirubicin; one of them received postoperative radiotherapy as well. All 5 patients are alive and disease-free for a period ranging from 7 to 26 years.

Conclusion: The excellent prognosis of those rare cases with multiple primary neuroblastomas elucidates questions concerning the tumor's biological behavior and the role of different factors affecting cell differentiation, tumor growth and dissemination.