Diagnosis and Management of Pheochromocytoma in a Resource-Challenged Setting: A Case Report
A case report
Phaeochromocytoma(PCC) is a rare catecholamine- secreting tumor, derived from chromaffin cells of the sympathetic nervous system. Phaechromocytomas are exceptionally rare in children, and are responsible for about 1% of pediatric hypertensive patients . They arise most commonly in the adrenal glands (~85% of tumours) but may also arise from chromaffin cells of the sympathetic nervous system, when they are known as paragangliomas (PGLs).
The tumors are more common in boys, with a 2:1 ratio of boys to girls in pediatric PCC/PGL cohorts. PCC/PGL often occur as sporadic tumors, but may occur sometimes as part of hereditary tumor syndromes, chiefly Von Hippel Lindau (VHL) disease and multiple endocrine neoplasia (MEN) 2A and 2B . About 50% of cases are missed, and discovered at autopsy because the diagnosis was not considered at presentation.
We report this case of phaechromocytoma to draw the attention of clinicians in our environment to this unusual cause of paediatric hypertension, and the need for a thorough investigation for the aetiology hypertension in children. We also underline the fact that this feared tumour can be successfully managed even in areas with limited resources such as ours. Finally, we encourage physicians to measure blood pressures of children presenting to their clinics.