Ends of the spectrum best practices for early detection and multidisciplinary management of acromegaly.

IF 3.2 2区 医学 Q2 CLINICAL NEUROLOGY Journal of Neuro-Oncology Pub Date : 2024-11-28 DOI:10.1007/s11060-024-04833-w
Stephanie Kim Cheok, Samon Tavakoli-Sabour, Ryan T Beck, Nathan Zwagerman, Adriana Ioachimescu
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Abstract

Purpose: 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.

Methods: 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.

Results: 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.

Conclusions: 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.

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肢端肥大症早期检测和多学科管理的最佳实践。
目的:肢端肥大症的特点是临床表现隐匿和诊断延迟。延误时间越长,合并症越多,在治疗分泌生长激素的垂体腺瘤(GH-PA)后,合并症可能持续存在。手术是治疗分泌生长激素的垂体腺瘤的主要方法,可使病情得到持久缓解。然而,约 50%的患者在术后需要接受药物治疗。在达到生化控制后,存活率会恢复正常。这篇微型综述将讨论肢端肥大症的两端挑战,包括延迟诊断、残留肿瘤的管理和持续的合并症:我们综合了相关文献,并介绍了一例肢端肥大症病例,该病例凸显了诊断和治疗顽固性肢端肥大症的临床决策的复杂性:结果:尽管生化检测方法有所改进,但大多数肢端肥大症患者平均在最初出现症状五年后才被确诊。高怀疑指数不仅取决于肢端肥大,还取决于提示肢端肥大症的一系列表现和合并症。生化疾病持续存在的患者需要接受药物治疗。体生长抑素受体配体是药物治疗的基石,可单独使用,也可与多巴胺激动剂和生长激素受体拮抗剂联合使用。通过改进药物治疗方案和仔细考虑合并症,可以对患者进行个体化管理。如果药物治疗后肿瘤仍有进展,则应考虑再次手术和放射治疗。在罕见的耐药和侵袭性肿瘤病例中,需要神经肿瘤学的专业知识:结论:通过针对肢端肥大症多方面临床表现的教育提高人们的认识,可缩短诊断和治疗时间。由专家进行多学科管理可提高生化控制和肿瘤控制的可能性。
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来源期刊
Journal of Neuro-Oncology
Journal of Neuro-Oncology 医学-临床神经学
CiteScore
6.60
自引率
7.70%
发文量
277
审稿时长
3.3 months
期刊介绍: The Journal of Neuro-Oncology is a multi-disciplinary journal encompassing basic, applied, and clinical investigations in all research areas as they relate to cancer and the central nervous system. It provides a single forum for communication among neurologists, neurosurgeons, radiotherapists, medical oncologists, neuropathologists, neurodiagnosticians, and laboratory-based oncologists conducting relevant research. The Journal of Neuro-Oncology does not seek to isolate the field, but rather to focus the efforts of many disciplines in one publication through a format which pulls together these diverse interests. More than any other field of oncology, cancer of the central nervous system requires multi-disciplinary approaches. To alleviate having to scan dozens of journals of cell biology, pathology, laboratory and clinical endeavours, JNO is a periodical in which current, high-quality, relevant research in all aspects of neuro-oncology may be found.
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