Clinical Characteristics of Pheochromocytoma in Korean Children / 대한소아내분비학회지

ABSTRACT

PURPOSE: Pheochromocytoma is a catecholamine-producing tumor that arises from the chromaffin cells of the adrenal medulla or the sympathetic ganglia. This study was undertaken to evaluate the clinical profiles of children with pheochromocytoma. METHODS: We retrospectively reviewed the clinical, radiological and outcome of 16 children who had been diagnosed as pheochromocytoma from May, 1984 to July, 2006 in the department of pediatrics, Seoul National University Hospital. RESULTS: The mean age at diagnosis was 12.3+/-3.4 years (range, 7.1-20 yrs). The male to female ratio was 10:6. The major symptoms were hypertension in 87.5%, sweating in 75%, tachycardia in 68.8% and headache in 62.5%. The sensitivities of 24 hour urinary norepinephrine and metanephrine were 100 %. All tumors were localized by combined study of CT, ultrasonography, MRI or (123)I-MIBG scan. Single (123)I- MIBG scan had sensitivity as 92.9%. Sporadic cases were 13 (81.3%). Bilateral pheochromocytomas were observed in 6 patients (37.5%). The pheochromocytoma developed in 6 patients with underling disease; von Hippel-Lindau (VHL) syndrome (3), neurofibromatosis (1), and cyanotic congenital heart diseases (2). Adrenalectomy was performed in 13 patients, and tumorectomy was in 3 patients. Two patients were diagnosed as malignant pheochromocytoma. CONCLUSION: Because the symptoms are deceptive and variable, the hormonal and radiological study should be performed carefully in suspected cases. The pheochromocytoma can now be operated safely. The tumor might present as a component of other syndromes such as multiple endocrine neoplasia type 2, neurofibromatosis, and VHL syndrome. Thus comprehensive consideration is needed at initial diagnostic approach.