[Bilateral pheochromocytomain a child with atypical primary presentation; a very rare case]

ABSTRACT

Pheochromocytoma is a rare cathecolamine-producing tumor that arises from chromaffin cells of the adrenal medulla or extraadrenal paraganglionic tissues, and has different and variant presentations. Only 10% of ph eochromocytomas occur in children with an incidence of approximately 1 case per 100,000 patient-years. It may be completely asymptomatic, or present with symptoms and signs such as headache, dizziness, palpitation, diaphoresis or hyperhydrosis, and hypertension. Bilateral pheochromocytoma-specially in children-is very rare and almost familial, but it has few sporadic cases. The diagnosis of isolated bilateral pheochromocytoma depends on ruling out of congenital etiologies such as type 2 Multiple Endocr ine Neoplasia [MENII], Von Hippel Lindau disease [VHL], type 1 Neurofibromatosis [NF I], Tuberous Sclerosis [TS], and Sturge Weber. The patient was a 12 year old boy who was referred with unusual suspicious symptoms of pheochromocytoma [weakness, dizziness, and hyperhydrosis] since the last year. During the disease, he had episodic headaches without hypertension. He had not any problem or disease until one year before. Hematologic exam revealed an elevated Erythrocyte Sedimentation Rate. In renal sonography and scan, we had hydonephrosis and pyelocaliceal dilatation.The collected 24hour urine contained elevated load of cathecolamine methabolites. Abdominal CT showed solid enhanced tumors in both adrenals, confirmed by MIBG scan. The patient was treated with bilateral adrenalectomy and diagnosis was confirmed by pathology and IHC staining. Although most pheochromocytomas have typical presentations, they may present with unusual symptoms and signs like the present case. So we should consider pheochromocytoma in patients with familial history of the disease, patients with typical symptoms or hypertension, and also patients with unusual presentations