A Case of Pheochromocytoma

Background: Pheochromocytoma is a rare neuroendocrine tumor that arises from the chromaffin cells, originates in the adrenal gland, particularly in the medulla.
Pheochromocytomas can produce large quantities of catecholamines (such as epinephrine and norepinephrine) causing severe hypertension.

The case: A 6-year-old male presented with a 2 months history of mood swings associated with severe headaches, facial flushing, sweating, increased water intake and urination. CT scan of abdomen and pelvis without contrast revealed a 2.5x2.8x3.1cm heterogeneous right adrenal mass. Patient was admitted to the pediatric intensive care unit due to severe hypertension and was started on Nicardipine drip and phenoxibenzamine.
Initial studies revealed a 24-hour urine Vanillylmandelic acid of 21.6 mg/day (Ref. Range 0.0-7.5), 24-hour urine Normetanephrine was 7059 ug/24 hrs (Ref. Range 13-252), urine Normetanephrine was 5679 pg/ml (Ref. range 10-25) and urine Metanephrine was 57 pg/ml (Ref. range 2-15).
Subsequent imaging studies revealed an MRI of the abdomen that showed heterogeneously enhancing 3.2 cm right suprarenal mass and no liver lesion is identified however MIBG Scintigraphy showed tracer uptake in the right upper quadrant corresponding with adrenal mass seen by CT, compatible with pheochromocytoma, additionally a smaller focus of mild uptake within hepatic segment. Chromosomal microarray was sent to rule out an underlying genetic disease .

The patient underwent surgery to remove the tumor , pathology confirmed pheochromocytoma.
Patient remained stable after surgery he has been off antihypertensive medication.

Discussion: Pheochromocytoma are exceedingly rare in children. Phechromocytoma has been associated several genetic syndromes such as in multiple endocrine neoplasia, Type I1 (MEN-11), Neurofibromatosis (Von Recklinghausen’s disease), Von Hippel Lindau syndrome and cerebellar hemangioblastomas therefore, genetic work up is essential.

Most of these tumors do not spread and are cured with surgery, however in rare cases it can cause metastasis. Recurrence is unusual but patients need to be monitored lifelong.