소아 청소년기에 발생한 뇌하수체 선종 24 증례의 임상상과 치료경과
Childhood Onset Pituitary Adenoma: Clinical Presentations and Outcomes of 24 Cases
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목적: 본 연구에서는 소아 청소년기의 기능성 및 비기능성 뇌하수체 선종 환자들에서 진단 당시 임상양상과 치료 경과에 대해 알아보고자 하였다. 방법: 18세 미만에 진단된 뇌하수체 선종 환자들의 의무기록을 후향적으로 조사하였다. 결과: 총 24명(남성 9명) 환자의 진단시 평균 연령은 12.3세였고, 추적관찰기간은 평균 4.5년이었다. 뇌하수체 선종은 PRL 분비 선종(7명, 29.2%), ACTH 분비 선종(4명, 16.7%), GH 분비 선종(3명, 12.5%) 및 비기능성 선종(10명,41.7%)으로 분류하였다. 거대 선종(13명, 54.2%)과 미세 선종(11명, 45.8%)의 비율은 비슷하였다. 모든 PRL 분비선종을 포함한 기능성 선종 환자들과 비기능성 선종 2명을 포함하여 16명(66.7%)의 환자에서 종양 압박 증상으로 수술을 받았다. 뇌하수체 수술을 받은 환자들은 수술을 받지 않은 환자들에 비해 뇌하수체 호르몬 결핍의 위험이 컸다(75.0% vs. 12.5%, P =0.008). 재발한 5명의 환자는 모두 거대선종 수술 후 잔존 종양이 남은 환자였다. 마지막 추적당시의 비만 정도는 진단시 비만도에 영향을 받았다(P＜0.001). 결론: 본 연구에서는 우연종으로 발견된 경우가 많아 비기능성 선종의 빈도가 기존의 보고에 비해 높았고, 기능성 선종 중에서는 PRL 분비 선종이 가장 많았다. 감별 진단 및 치료 계획의 수립에 있어 호르몬 과잉 증상의 여부 및 호르몬분비 상태에 대한 주의깊은 평가가 중요하다. 또한, 특히 뇌하수체 수술을 시행 받은 뇌하수체 선종 환자에서 재발이나호르몬 결핍 여부에 대한 정기적인 추적관찰이 필수적이다.
Abstract
Purpose: Our purpose was to evaluate the clinical presentations at diagnosis and treatment outcomes of functional pituitary adenoma (PA) and nonfunctional PAs (NFPA)in childhood and adolescence. Methods: We performed a retrospective review of the medical records of patients diagnosed with PA before 18 years of age. Results: The mean age at diagnosis of 24 patients (9 males, 15 females) was 12.3years, and the mean follow-up period was 4.5 years. PAs were categorized as follows:prolactinoma (n=7, 29.2%), adrenocorticotropic hormone secreting adenomas (n=4,16.7%), growth hormone secreting adenomas (n=3, 12.5%), and NFPAs (n=10, 41.7%). The proportion of macroadenoma (n=13, 54.2%) was similar to that of microadenoma (n=11, 45.8%). Sixteen patients (66.7%) who were diagnosed with functional PA (including all prolactinomas), and two NFPAs underwent surgery due to mass effects. The patients who received surgery had a higher risk for hypopituitarism than those who did not (75.0% vs. 12.5%; P =0.008). Recurrence occurred in five patients, all of whom had residual tumors after surgery for macroadenoma. The degree of obesity at latest follow-up was associated with that at diagnosis (P <0.001). Conclusion: The prevalence of NFPAs was higher than that of previous reports, which was explained by higher detection of incidentalomas. Prolactinoma was the most common functional PA. Thorough investigation of symptoms related to hormone excess and hormone profiles is important for differential diagnosis and decision regarding treatment plan. Additionally, the regular follow up for relapse and endocrine deficit is mandatory in patients with PA, especially those who had received pituitary surgery.
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