Arosio M, Cannavò S, Epaminonda P, Ronchi C, Chiodini I, Adda G
Institute of Endocrine Sciences, University of Milan, Milan, Italy.
J Endocrinol Invest. 2003;26(10 Suppl):36-43.
This short review summarizes the results of treatments now available in Italy for the management of GH and IGF-I excess due to primary pituitary somatotroph adenoma, which accounts for over 99% of cases of acromegaly. Goals of treatment of acromegaly should now include, in addition to the reduction of tumor bulk and symptomatic relief, the lowering of GH circulating concentrations to below a critical level (2.5 microg/l, "safe" GH), the normalization of serum IGF-I concentrations according to age, improvement (or at least not worsening) of co-morbidities (diabetes mellitus, hypertension, cardiomyopathy, sleep-apnea), the decrease of the risk of premature mortality. Surgery, radiation (fractionated conventional radiotherapy and radiosurgery) and medical treatments with dopamine agonists and somatostatin analogs are the available options that are discussed in detail. The treatment of acromegaly must be tailored to the needs of the individual patient. Age, tumor size and invasiveness, GH concentrations, the patient's general medical conditions, presence and severity of co-morbidities, availability of local resources such as an expert neurosurgeon or gamma-knife radiosurgery, and of course the informed wishes of the patient are all factors that must be taken into account. For most patients the treatment will be multimodal. However, despite criteria and guidelines based on continuously emerging information about the management of acromegaly, patient outcomes are still less than desirable, with 10 to 20% of patients with uncontrolled disease, despite the use of all available therapies. This underscores the need for the quick introduction in clinical practice of the new therapies.
本简短综述总结了目前意大利可用于治疗因原发性垂体生长激素细胞腺瘤导致的生长激素(GH)和胰岛素样生长因子-Ⅰ(IGF-Ⅰ)过多的治疗结果,原发性垂体生长激素细胞腺瘤占肢端肥大症病例的99%以上。肢端肥大症的治疗目标现在应包括,除了缩小肿瘤体积和缓解症状外,将循环中的GH浓度降低至临界水平以下(2.5微克/升,“安全”GH),根据年龄使血清IGF-Ⅰ浓度正常化,改善(或至少不恶化)合并症(糖尿病、高血压、心肌病、睡眠呼吸暂停),降低过早死亡风险。手术、放疗(分次常规放疗和放射外科)以及使用多巴胺激动剂和生长抑素类似物的药物治疗是详细讨论的可用选择。肢端肥大症的治疗必须根据个体患者的需求进行调整。年龄、肿瘤大小和侵袭性、GH浓度、患者的一般医疗状况、合并症的存在和严重程度、当地资源(如专家神经外科医生或伽玛刀放射外科)的可用性,当然还有患者的知情意愿,都是必须考虑的因素。对于大多数患者,治疗将是多模式的。然而,尽管基于不断涌现的肢端肥大症管理信息制定了标准和指南,但患者的治疗结果仍不尽人意,尽管使用了所有可用疗法,仍有10%至20%的患者疾病未得到控制。这凸显了在临床实践中快速引入新疗法的必要性。