Cheok Stephanie Kim, Tavakoli-Sabour Samon, Beck Ryan T, Zwagerman Nathan, Ioachimescu Adriana
Department of Neurosurgery, Medical College of Wisconsin, Hub for Collaborative Medicine, 8701 Watertown Plank Rd., Milwaukee, Wisconsin, 53226, USA.
Department of Radiology, Division of Neuroradiology, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.
J Neurooncol. 2025 Jan;171(1):1-9. doi: 10.1007/s11060-024-04833-w. Epub 2024 Nov 28.
Acromegaly is characterized by an insidious clinical presentation and delayed diagnosis. Longer delays are associated with more comorbidities which can persist after treatment of the growth hormone-secreting pituitary adenoma (GH-PA). Surgery is the primary therapy of GH-secreting PA, which can lead to durable remission. However, approximately 50% of patients require medical treatment postoperatively. Survival normalizes after achieving biochemical control. This mini-review will address ends of the spectrum challenges in acromegaly, including delayed diagnosis and management of the residual tumor and persistent comorbidities.
We synthesize relevant literature and present a case of acromegaly that highlights the complexity of clinical decision-making in the diagnosis and treatment of persistent acromegaly.
Despite improved biochemical assays, most patients with acromegaly are diagnosed on average five years after initial symptoms. A high index of suspicion does not rely exclusively on acral enlargement, but also a constellation of manifestations and comorbidities suggestive of acromegaly. Medical therapy is required in patients with persistent biochemical disease. Somatostatin receptor ligands are the cornerstone of medical treatment and can be used alone or in combination with dopamine agonists and growth hormone receptor antagonists. Improved options of medical treatment and careful consideration of comorbidities enables individualized patient management. Reoperation and radiation are considered for tumor progression despite medical therapy. In rare cases of resistant and aggressive tumors, neuro-oncology expertise is required.
Increased awareness through education targeting the multifaceted clinical presentation of acromegaly shortens the time to diagnosis and treatment. Multidisciplinary management by specialists increases the likelihood of biochemical and tumor control.
肢端肥大症的临床表现隐匿,诊断往往延迟。诊断延迟时间越长,合并症越多,这些合并症在生长激素分泌型垂体腺瘤(GH-PA)治疗后可能持续存在。手术是分泌GH的垂体腺瘤的主要治疗方法,可实现持久缓解。然而,约50%的患者术后需要药物治疗。实现生化控制后生存率恢复正常。本综述将探讨肢端肥大症在诊断和治疗方面的极端挑战,包括诊断延迟、残留肿瘤的管理和持续存在的合并症。
我们综合了相关文献,并介绍了一例肢端肥大症病例,该病例突出了持续性肢端肥大症诊断和治疗中临床决策的复杂性。
尽管生化检测方法有所改进,但大多数肢端肥大症患者在出现初始症状后平均五年才被诊断出来。高度怀疑不仅依赖于肢端肥大,还包括一系列提示肢端肥大症的临床表现和合并症。生化指标持续异常的患者需要药物治疗。生长抑素受体配体是药物治疗的基石,可单独使用或与多巴胺激动剂和生长激素受体拮抗剂联合使用。更好的药物治疗选择以及对合并症的仔细考虑有助于实现个体化的患者管理。尽管进行了药物治疗,但肿瘤进展时仍考虑再次手术和放疗。在罕见的耐药性和侵袭性肿瘤病例中,需要神经肿瘤学专家的专业知识。
通过针对肢端肥大症多方面临床表现的教育提高认识,可缩短诊断和治疗时间。专家进行多学科管理可提高生化指标控制和肿瘤控制的可能性。