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TÜRKÇE
Poster - 281
Pediatric Pheochromocytoma and Paraganglioma: A 15-Year Single-Center Experience
Omar Zeinedine 1, Marcela Pychova 1, Karel Pycha 1, Aleš Vicha 2, Marek Schmidt 3, Jana Pavlickova 4, Michal Rygl 1
1 Department of Pediatric Surgery, 2nd Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czech Republic
2 Department of Pediatric Oncology and Hematology, 2nd Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czech Republic
3 Urology Clinic, Charles university and Motol University Hospital, Prague, Czech Republic
4 Department of Anesthesiology and Intensive Medicine, 2nd Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czech Republic
Introduction: Pheochromocytoma and paraganglioma (PPGL) are rare neuroendocrine tumors that secrete catecholamines. This study reviews our experience with the diagnosis and management of these tumors in pediatric patients.
Materials and Methods: We conducted a retrospective analysis of pediatric patients who underwent surgery at Motol University Hospital between 2009 and 2024.
Results: A total of 18 patients (8 boys and 10 girls) were treated, with a median age of 14.2 years (range, 9.7–18.7 years). The most common presenting symptoms were hypertension (n=15), headaches (n=8), sweating (n=8), and palpitations (n=5); All patients underwent genetic testing. (VHL) Von Hippel-Lindau syndrome was confirmed in three patients, all of whom exhibited bilateral disease (one synchronous, two metachronous). Two patients were found to have a SDHB (Succinate dehydrogenase type B) mutation. Histopathological analysis revealed 13 cases of pheochromocytoma (with bilateral involvement in two patients) and six cases of paraganglioma. Surgical management of pheochromocytomas included adrenalectomy performed via open (n=6) and laparoscopic (n=7) approaches, with tumor resection preserving adrenal tissue in one patient with bilateral disease. All paragangliomas were resected via laparotomy, with one case managed laparoscopically.
The median follow-up duration was 8.6 years (range: 8 months to 15.9 years). Postoperatively, clinical symptoms resolved gradually in all patients. No deaths were reported during the follow-up period.
Conclusion: Pheochromocytoma and paraganglioma should be considered in the differential diagnosis of retroperitoneal tumors and in children with hypertension. Surgical resection remains the definitive treatment. Patients with confirmed genetic mutations (SDHB and VHL) are at a high risk for recurrence and require lifelong follow-up.