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Paediatric Pituitary Adenomas

医学 尿崩症 肢端肥大症 儿科 垂体腺瘤 队列 回顾性队列研究 放射治疗 经蝶手术 垂体瘤 内科学 生长激素 腺瘤 激素
作者
Satyam Singh Jayant,Rimesh Pal,Ashutosh Rai,Kirti Gupta,Bishan Dass Radotra,Rama Walia,Sivashanmugam Dhandapani,Manjul Tripathi,Chirag Ahuja,Prakamya Gupta,Anil Bhansali,Liza Das,Pinaki Dutta
出处
期刊:Neurology India [Medknow]
卷期号:70 (1): 304-311 被引量:10
标识
DOI:10.4103/0028-3886.338667
摘要

Paediatric pituitary adenomas (PPAs) are uncommon, with evidence confined to small cohorts.We aimed to elucidate the baseline profile and outcomes of PPAs in a large, contemporary, monocentric cohort.Pituitary clinic at PGIMER over 8 years (2010-2018).PPAs in patients (≤20 years at diagnosis) were included. A retrospective review of their baseline clinico-biochemical and radiological profiles and outcomes post pituitary surgery/medical management was performed.There were a total of 74 patients, of which 42 were female. The median age was 15 (IQR 13-18) years. Corticotropinomas (32.4%) and somatotropinomas (25.7%) were common, with 1 case of TSHoma and pituitary blastoma. The most common presentation was headache (57%) overall and menstrual irregularities (64.2%) in girls. Most (78%) had macroadenomas. Prolactinomas showed an excellent response to primary medical therapy (83.3%). Transsphenoidal surgery was performed in 81% of patients. Diabetes insipidus (30%) and hyponatremia (26.7%) emerged as common postoperative complications. Adjuvant medical management was required in 25%, and radiotherapy in 18%. Remission rates in Cushing's and acromegaly were 62.5% and 57.8%, respectively, with long-term hormone deficits noted in one-third of patients.PPAs have unique features and management challenges, including effects on growth and puberty. Functional tumours and macroadenomas are common. Remission can be achieved in more than half of the patients, with endocrine deficits persisting in about a third of cases, needing long-term surveillance.

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