Pheochromocytoma – Quick revision card

Neuroendocrine tumor Pheochromocytoma( PCC ) -are neuroendocrine tumors of the adrenal medulla ( chromaffin cell )

Paraganglion -from the sympathetic and parasympathetic ganglion (PGL )

incidence ranges between 2 and 8 per million picked up incidentally and of them 80-85 % are PCCs and 15 % to 20 % are PGLs in out patient clinics these occur in 0.1 % and 0.6 % of patients with hypertension they can appear at any age but more common in 3rd to 5th decade hereditary disease is more likely to manifest in younger patients

PCC's mostly arise from the adrenal medulla whereas PGLs tend to arise from sympathetic or parasympathetic ganglia outside the of the adrenal medulla for e.g retroperitoneum , pelvis , thorax , head and neck

Up to 70 % of PPGLs are caused by germline or somatic variants in one of the the susceptibility gene ( ie a hereditary tumor ) More than 20 mutations are known

Symptoms can manifest due to excessive production of catecholamines ( epinephrine , norepinephrine and dopamine )

Hypertensive crisis palpitations ,diaphoresis , headache ( classic triad ) + hypertension due to massive release of catecholaminergic hormones from the tumor ( and resultant haemodynamic and...