Neuroendocrine Unit and Neuroendocrine and Pituitary Tumor Clinical Center, Massachusetts General Hospital, Boston.
Neuroendocrine Unit and Neuroendocrine and Pituitary Tumor Clinical Center, Massachusetts General Hospital, Boston; Harvard Medical School, Boston, MA.
Mayo Clin Proc. 2022 Feb;97(2):333-346. doi: 10.1016/j.mayocp.2021.11.007.
Acromegaly is typically caused by a growth hormone-secreting pituitary adenoma, driving excess secretion of insulin-like growth factor 1. Acromegaly may result in a variety of cardiovascular, respiratory, endocrine, metabolic, musculoskeletal, and neoplastic comorbidities. Early diagnosis and adequate treatment are essential to mitigate excess mortality associated with acromegaly. PubMed searches were conducted using the keywords growth hormone, acromegaly, pituitary adenoma, diagnosis, treatment, pituitary surgery, medical therapy, and radiation therapy (between 1981 and 2021). The diagnosis of acromegaly is confirmed on biochemical grounds, including elevated serum insulin-like growth factor 1 and lack of growth hormone suppression after glucose administration. Pituitary magnetic resonance imaging is advised in patients with acromegaly to identify an underlying pituitary adenoma. Transsphenoidal pituitary surgery is generally first-line therapy for patients with acromegaly. However, patients with larger and invasive tumors (macroadenomas) are often not in remission postoperatively. Medical therapies, including somatostatin receptor ligands, cabergoline, and pegvisomant, can be recommended to patients with persistent disease after surgery. Select patients may also be candidates for preoperative medical therapy. In addition, primary medical therapy has a role for patients without mass effect on the optic chiasm who are unlikely to be cured by surgery. Clinical, endocrine, imaging, histologic, and molecular markers may help predict the response to medical therapy; however, confirmation in prospective studies is needed. Radiation therapy is usually a third-line option and is increasingly administered by a variety of stereotactic techniques. An improved understanding of the pathogenesis of acromegaly may ultimately lead to the design of novel, efficacious therapies for this serious condition.
肢端肥大症通常由生长激素分泌垂体腺瘤引起,导致胰岛素样生长因子 1 过度分泌。肢端肥大症可能导致多种心血管、呼吸、内分泌、代谢、肌肉骨骼和肿瘤合并症。早期诊断和充分治疗对于减轻与肢端肥大症相关的过度死亡率至关重要。使用关键词“生长激素、肢端肥大症、垂体腺瘤、诊断、治疗、垂体手术、药物治疗和放射治疗”(1981 年至 2021 年)在 PubMed 上进行了搜索。肢端肥大症的诊断基于生化依据得到确认,包括血清胰岛素样生长因子 1 升高和葡萄糖给药后生长激素抑制缺乏。建议在肢端肥大症患者中进行垂体磁共振成像,以确定潜在的垂体腺瘤。经蝶窦垂体手术通常是肢端肥大症患者的一线治疗方法。然而,较大和侵袭性肿瘤(大腺瘤)的患者术后通常无法缓解。对于手术后持续存在疾病的患者,可以推荐使用生长抑素受体配体、卡麦角林和培维索孟等药物治疗。选择合适的患者也可能是术前药物治疗的候选者。此外,对于没有对视交叉产生肿块效应且不太可能通过手术治愈的患者,原发性药物治疗也有作用。临床、内分泌、影像学、组织学和分子标志物可帮助预测药物治疗的反应;然而,需要前瞻性研究来证实。放射治疗通常是三线选择,并且越来越多地通过各种立体定向技术进行。对肢端肥大症发病机制的深入了解最终可能会为这种严重疾病设计出新型、有效的治疗方法。