Pheochromocytomas and paragangliomas are uncommon in the paediatric population. Tumours arising from adrenal medulla are pheochromocytomas, extra-adrenal or nonfunctionaltumors are paragangliomas whereas functional tumors are referred to as extra-adrenal pheochromocytomas. In children, pheochromocytoma is more frequently associated with other familial syndromes (neurofibromatosis) or as a component of MEN syndromes.

1. To report a series of three cases of pheochromocytoma in paediatric age group 2. To review the literature on pheochromocytoma in children.

We are reporting three rare cases of pheochromocytoma in paediatric age group at Niloufer Hospital for Women and Children a 600 bedded tertiary care centre for women and children

A 7yr old and two 12 years-old boys presented with sustained hypertension, headache, sweating, and visual blurring. 24 hour urinary catecholamines, Ultrasonography and computed tomography were diagnostic. The first child presented as a left infra-renal extra-adrenal para-aortic mass and the other two as left adrenal masses. Pre-op optimization was done with a combination of alpha, beta and calcium channel blockers. Intra operative monitoring included ECG, SpO2, EtCO2, urine output along with CVP and IBP. Haemodynamic stabilization was achieved with thorough titration of beta blockers, NTG & vasopressors

Meticulous pre-operative optimization, good intra-operative monitoring and proper post-operative care helped in normalization of pressures after surgical resection

Pre-intra-and postoperative care is as important as the surgical procedure in the management of pheochromocytoma.

Wang X, Nine cases of childhood adrenal tumour presenting with hypertension and a review of the literature ActaPaediatr 2007 Jun; 96(6):930-4