Pheochromocytoma and Paraganglioma in Pediatric Age

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Introduction: Pheochromocytoma (PHE) and paraganglioma (PGL) are rare tumors of the adrenal medulla and extra-adrenal sympathetic chromaffin tissue. Nearly 30% of them are familiar or associated with genetic syndromes (VHL, MEN2, NF1, SDH mutation, Pacak-Zhuang). Only 20% PHE occur before 20 years of age. Incidence of pediatric PHE is 0.3:1 million.

Aim(s): Fifteen years of experience at a single academic institution. Symptoms, clinical outcome, definition of risk factors and age-specific differences of PHE/PGL.

Materials and methods: During the 15-year period, we diagnosed four patients with PHEO/PGL (0.2% of all tumors), median age 16 years (7–17). The main clinical symptom was headache, sustained hypertension, abdominal pain and weakness. Duration of symptoms 2 days-17 months. All patients had localized disease. A 7-year-old boy developed PHE in the contralateral adrenal gland. None of the patients received adjuvant therapy.

Conference: 11th Annual ENETSConcerence (2014)

Presenting Author: Bajciova V

Authors: Bajciova V, Kren L, Vicha A,

Keywords: pheochromocytoma, paraganglioma, pediatric age,