Endocrine Abstracts

Pheochromocytomas and paragangliomas, tumors originating from the chromaffin cells, are rare in children.

We report an 8-year-old boy who was admitted to the intensive care unit with seizures for which the child had to be intubated, severe hypokalemia (1.8 mEq/l), hyponatremia (127 mEq/l) and fever. Parents reported that several months before admission the boy had nocturnal sweating. Brain MRI revealed areas of increased sign intensity in the parietal lobes bilaterally between the cortex and the subcortical region. Blood thyroid hormone levels were normal. He was initially treated as encephalitis and several boluses of potassium chloride were administered and the serum levels of sodium and potassium returned to normal, without fluid restriction. The child showed remarkable improvement in 48 hours. During hospitalization hypertension was diagnosed (180/95 mmHg) and the child complained for headaches, palpitations, polydipsia, polyouria and nocturnal sweatings. He was treated with combination of dihydralazine, felodipine, enalaprile and propranolole but without blood pressure control. Urinary 24-hour catecholamines (6440microg, normal range 14–108) and normetanephrines (19222microg normal range 88–444) were markedly elevated. Serum levels of renin (49.4 microIU/ml, normal range 3.3–41) and aldosterone (37.7 ng/dl, normal range 3–28) were elevated. Abdomen MRI showed a mass (4×4.5×3 cm) in the left paraspinal area pushing down left kidney. Whole body MIBG I-131 scan was negative. The antihypertensive therapy was modified to phenoxybenzamine followed by propranolole with normalization of blood pressure. A laparotomy with removal of the retroperitoneal mass was perfomed. The intraoperative course was uneventful. Histologically the mass proved a well-demarcated paraganglioma. No infiltration of nearby structures or other malignant features were noted. Postoperatively, the child was asymptomatic, blood pressure and urinary catecholamines returned to normal. Genetic testing of VHL, SDHB, SDHD and RET genes was recommended.