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Long term survival in five rare cases with multiple primary neuroblastomas
O Brankov1, I Christosova, L Marinova
1Department of Pediatric Surgery, University Hospital N.I. Pirogov, Sofia, Bulgaria. brankov@pedsurg.net
Journal of B.U.ON. : Official Journal of the Balkan Union of Oncology
|February 20, 2007
Summary
Multiple primary neuroblastomas are rare in infants but have an excellent prognosis with successful treatment. This study highlights 5 cases of multifocal neuroblastomas, all surviving disease-free after surgery and chemotherapy.
Area of Science:
- Pediatric Oncology
- Surgical Oncology
- Developmental Biology
Background:
- Multiple primary neuroblastomas, originating from the sympathetic nervous system, are exceptionally rare in infants.
- This study presents a case series from Bulgaria, representing the largest clinical material on this condition in the region.
Observation:
- Five cases of multifocal neuroblastomas were identified among 193 neuroblastoma patients treated over 26 years (2.6% prevalence).
- All affected patients were male, diagnosed between 2 months and 4 years of age.
- The tumors were multifocal, arising in different sympathetic nervous system locations.
Findings:
- All five patients achieved successful treatment outcomes through radical surgery.
- Four patients received adjuvant chemotherapy (vincristine, cyclophosphamide, epirubicin), and one also had radiotherapy.
- All patients remain alive and disease-free, with follow-up periods ranging from 7 to 26 years.
Implications:
- The excellent prognosis in these rare multifocal neuroblastoma cases offers insights into tumor biology.
- Understanding factors influencing cell differentiation, tumor growth, and dissemination is crucial for managing rare pediatric cancers.
- This study underscores the effectiveness of multimodal treatment, including surgery and chemotherapy, for multifocal neuroblastomas.
