Diagnostic and treatment pitfalls of pheochromocytoma in children

Authors: P. Kunovský 1;  Z. Pribilincová 2;  A. Kriššáková 1;  R. Dinka 1;  M. Kováč 1;  J. Krbaťa 3;  J. Malina 4;  J. Babala 5
Authors‘ workplace: Národný ústav srdcových a cievnych chorôb, a. s., Detské kardiocentrum, JIS kardiologického oddelenia, Bratislava, primár MUDr. P. Kunovský, PhD., MBA 1;  2. detská klinika DFNsP a LFUK, Bratislava, prednosta prof. MUDr. L. Kovács, DrSc., MPH 2;  Rádiologické oddelenie DFNsP, Bratislava, primár MUDr. D. Haviar 3;  Klinika onkologickej chirurgie OUSA a LFUK, Onkologický ústav svätej Alžbety, s. r. o., Bratislava, prednosta prof. MUDr. Š. Durdík, PhD., MHA 4;  Klinika detskej chirurgie DFNsP a LFUK, Bratislava, prednosta doc. MUDr. J. Trnka, CSc. 5
Published in: Čes-slov Pediat 2015; 70 (4): 249-254.
Category: Education


Pheochromocytomas and paragangliomas (PHEO/PGL) are neuroendocrine tumors that arise from sympathetic and parasympathetic paraganglia. PHEO presents most frequently by permanent or paroxysmal hypertension, headache, sweating, palpitations and anxiety. Diagnosis is based on typical clinical presentation confirmed by biochemical measurement of plasma metanephrines and normetanephrines with the sensitivity almost up to 100% being the test of choice for the diagnosis of PHEO. The morphological test of choice is either computed tomography or magnetic resonance imaging, which have similar diagnostic sensitivities of the abdomen and pelvis. Definitive and causal treatment is by surgical resection and the procedure of choice for most PHEO is laparoscopic adrenalectomy. Main goal of preoperative management is normalization of blood pressure, heart rate, reinstitution of normovolemia and prevention of catecholamine „storm“ and its effects on cardiovascular system during manipulation with tumor in the course of operation. Authors suggest as initial treatment in hypertensive crisis intravenous urapidil and in case of persisting tachycardia the combination with metoprolol after adequate time delay to achieve satisfactory alpha blockade and/or calcium channel blocker amlodipine.

The aim of the report is to draw attention to difficulties in diagnostics of PHEO and to outline current approaches to preoperative management to prevent severe adverse events of children suffering from this tumor. The pediatric provider should be able to recognize and screen for such tumors, particularly in the context of a known genetic predisposition (von Hippel-Lindau syndrome, MEN type 2, neurofibromatosis type 1, and paragangliomas). The clinical presentation of PHEO might be sometime atypical so most important part of correct diagnosis depends on having a clinical suspicion for it and then confirming the diagnosis biochemically.

Key words:
pheochromocytoma, hypertensive crisis, von Hippel-Lindau syndrome, urapidil


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Neonatology Paediatrics General practitioner for children and adolescents
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