Exploring the Heterogeneity of Pediatric Neuroendocrine Tumors: Single-Center Experience
Authors' conclusion
Early diagnosis, genetic screening, and long-term follow-up are crucial in managing pediatric NETs to reduce morbidity and mortality. Genetic testing is essential for identifying comorbidities and screening family members at risk.
Study context
- Population
- Children diagnosed with neuroendocrine tumors (NETs) (PPGL, gastroenteropancreatic neuroendocrine tumors (GEP-NETs), medullary thyroid carcinoma (MTC), C-cell hyperplasia), 2000–202457.6% male
- Participants
- 26 children
- Outcome
- NET type distribution
Methodological detailssingle-centre
- Single-centre
Main finding
As reported in the abstract
- GEP-NETs
- 15.4%
- PPGL
- 15.4%
- C-cell hyperplasia
- 15.4%
- MTC
- 53.8%
- Median age at diagnosis
- 8.8 (range: 1.0-16.6) years
Observational data: associations, not effects.
Further findings
Genetic analysis
- RET gene mutations
- 69.2%
- VHL gene mutations
- 3.8%
- No mutations
- 3.8%
- No genetic testing
- 23%
Mortality · median follow-up 5 (range: 1-14) years
- Died due to MTC
- 1 patient
Original publication
Exploring the Heterogeneity of Pediatric Neuroendocrine Tumors: Single-Center Experience. Horm Res Paediatr. Published 20 May 2025. doi:10.
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