소아 및 청소년기의 뇌하수체선종

Abstract

Between 1989 and 1993, 13 patients with pituitary adenoma underwent operation/radiosurgery at Yonsei University Hospital before their 18th birthday. This comprises an incidence of 5.3%(13/247) of all pituitary adenoma patients who were treated at our institution during the study period. There were 7 boys and 6 girls ranging in age from 10.6 to 17.9 years(average, 14.9 years). The mean follow-up period was 22.4 months(7-55 months). Immunohistochemical study revealed 4 prolactin(PRL)-secreting, 3 growth hormone(GH)-secreting(PRL was also positive in one), 1 adrenocortical hormone(ACTH)-secreting, 1 thyroid stimulating hormone(TSH)-secreting, and 4 non-functioning adenomas. Nine of 13 patients(77%) presented with symptomatic endocrinopathy due to hormonal hypersecretion and/or delay in linear growth or onset of puberty. Microadenomas comprised one of nine functionng adenomas, and one of 4 nonfunctioning adenomas, or 15% overall. In 10 patients(77%), at least one pituitary hormonal axis was abnormal. Transsphenoidal and transcranial resections were employed in 8 and 4 patients, respectively. One patient with nonfunctioning microadenoma underwent gamma knife radiosurgery. Endoclinologically successful treatment was possible in 4 of 9 functioning adenomas(44%). Gross total resections confirmed by follow-up study were achieved in 2 of 3 nonfunctioning adenomas. One nonfunctioning microadenoma treated by radiosurgery showed no progression on follow-up study 20 months after radiosurgery. Seven patients(54%) were treated successfully with either operation or radiosurgery. Adjuvant therapy was applied to 5 patients. Overall tumor control was achieved in 91%. Long-term partial or full hormone replacement was necessary in 69%. It is concluded that early diagnosis, vigorous approach to complete cure, and more strict endocrinological management for sexual maturation and growth are mandatory in the management of pediatric pituitary adenomas.