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Insulinoma in pediatric tuberous sclerosis complex: a case report.
Katia Librandi1, Serena Grimaldi2, Silvia Catalano3
1Postgraduate School of Pediatrics, University of Turin, Turin, Italy.
Frontiers in Pediatrics
|August 21, 2023
Summary
Tuberous sclerosis complex (TSC) can cause seizures due to hypoglycemia from insulinomas. This report details the first pediatric case of TSC-associated insulinoma, highlighting the need for awareness.
Area of Science:
- Genetics and rare diseases
- Pediatric endocrinology
- Neurology
Background:
- Tuberous sclerosis complex (TSC) is a genetic disorder with varied symptoms, often including epilepsy.
- Seizures in TSC can be linked to hypoglycemia, potentially caused by insulinomas.
- The mTOR pathway is implicated in TSC-associated tumors, affecting cell metabolism.
Observation:
- A pediatric patient with TSC presented with intractable seizures.
- Hypoglycemia was identified as the cause of the seizures.
- A pancreatic insulinoma was diagnosed and surgically removed.
Findings:
- This is the first reported case of a pediatric patient with TSC and insulinoma.
- The patient experienced seizure freedom after insulinoma removal.
- The association between TSC and insulinoma, previously reported in 11 adults, is further supported.
Implications:
- Recurrent seizures in TSC patients may indicate underlying hypoglycemia and insulinoma.
- Increased awareness and research are crucial for managing TSC-associated insulinomas.
- Early diagnosis and treatment can improve seizure control and patient outcomes.
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