嗜酸细胞瘤是一种罕见疾病，患病率约为每百万人60例，女性稍多于男性，并且在成年人四十岁左右出现高峰期。由于症状缓慢进展的原因，临床诊断经常会被延迟数年。有多种临床标准可定义嗜酸细胞瘤：潜在起病的畸形综合征，与垂体肿瘤相关的症状（头痛、视觉障碍），一般体征（出汗、腕管综合征、关节疼痛等），疾病的并发症（骨骼肌肉系统、心血管系统、呼吸系统、口腔、代谢方面的合并症、甲状腺结节、结肠息肉等），或者有时伴有高泌乳素分泌（男性勃起功能障碍或女性月经紊乱）或同时存在质量因子引起的垂体功能减退症（疲劳和其他与垂体激素缺乏相关的症状）。生物学诊断最初是通过IGF-I水平升高和经口葡萄糖耐量试验GH抑制不良或GH平均水平升高的重复测量来确认。在确诊病例中，垂体MRI影像可以确定原因性肿瘤，以更好地确定治疗方案。在少数病例中，嗜酸细胞瘤可能与遗传易感性有关，特别是在年轻人或家族环境中发生时。一线治疗通常是立即或暂时性药物治疗后切除生长激素细胞瘤。如果手术治疗无效，通常会提出药物治疗方案。对于药物无效或无法耐受的患者，可以根据具体情况讨论适应症合理或立体定位放射治疗。嗜酸细胞瘤应由多学科团队管理，最好在专家中心（如罕见垂体疾病参考中心或技能中心）的支持下进行。版权所有 © 2023。 Elsevier Masson SAS 发布。

Acromegaly is a rare disease with prevalence of approximately 60 cases per million, slight female predominance and peak onset in adults in the fourth decade. Clinical diagnosis is often delayed by several years due to the slowly progressive onset of symptoms. There are multiple clinical criteria that define acromegaly: dysmorphic syndrome of insidious onset, symptoms related to the pituitary tumor (headaches, visual disorders), general signs (sweating, carpal tunnel syndrome, joint pain, etc.), complications of the disease (musculoskeletal, cardiovascular, pneumological, dental, metabolic comorbidities, thyroid nodules, colonic polyps, etc.) or sometimes clinical signs of associated prolactin hypersecretion (erectile dysfunction in men or cycle disorder in women) or concomitant mass-induced hypopituitarism (fatigue and other symptoms related to pituitary hormone deficiencies). Biological confirmation is based initially on elevated IGF-I and lack of GH suppression on oral glucose tolerance test or an elevated mean GH on repeated measurements. In confirmed cases, imaging by pituitary MRI identifies the causal tumor, to best determine management. In a minority of cases, acromegaly can be linked to a genetic predisposition, especially when it occurs at a young age or in a familial context. The first-line treatment is most often surgical removal of the somatotroph pituitary tumor, either immediately or after transient medical treatment. Medical treatments are most often proposed in patients not controlled by surgical removal. Conformal or stereotactic radiotherapy may be discussed on a case-by-case basis, especially in case of drug inefficacy or poor tolerance. Acromegaly should be managed by a multidisciplinary team, preferably within an expert center such as a reference or skill center for rare pituitary diseases.Copyright © 2023. Published by Elsevier Masson SAS.