E O Mamedova, E G Przhiyalkovskaya, S A Buryakina, E V Bondarenko, A M Lapshina, M Yu Pikunov, Zh E Belaya, G A Melnichenko
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引用次数: 0
摘要
肢端肥大症是一种由生长激素(GH)分泌过多引起的神经内分泌疾病。在大多数情况下,肢端肥大症的病因是产生生长激素的垂体瘤。异位性肢端肥大症则更为罕见。异位性肢端肥大症发生于产生生长激素释放激素(GHRH)的肿瘤或产生 GH 的垂体外肿瘤。GHRH分泌过多的主要来源是肺部或胰腺的神经内分泌肿瘤(NET)。异位肢端肥大症的治疗包括手术切除 GHRH 过度分泌的来源,如果无法选择手术,则使用体生长激素类似物、培维索胺、化疗、免疫疗法或放射疗法。前两个病例的临床症状轻微,第二个病例的异位肢端肥大症伴有原发性甲状旁腺功能亢进。在第三个病例中,异位肢端肥大症伴有垂体大腺瘤,手术切除肺NET后,肢端肥大症仍未缓解。在这三个病例中,肺 NET 都是在其他疾病的胸部放射检查中偶然发现的。
[Ectopic acromegaly due to bronchial neuroendocrine tumors: the first description in Russia of three clinical cases].
Acromegaly is a neuroendocrine disorder caused by excessive production of growth hormone (GH). In the majority of cases the cause of acromegaly is a pituitary tumor producing GH. Cases of ectopic acromegaly are much rarer. Ectopic acromegaly occurs in cases of tumors which produce growth hormone-releasing hormone (GHRH) or extrapituitary tumors which produce GH. The main sources of excessive GHRH production are neuroendocrine tumors (NETs) of the lung or pancreas. Treatment of ectopic acromegaly consists of surgical removal of the source of GHRH hyperproduction and in cases where surgery is not an option, somatostatin analogues, pegvisomant, chemotherapy, immunotherapy or radiation therapy are used.In this article three cases of ectopic acromegaly due to GHRH-producing lung NETs are presented, each of them being notable for a number of features. In the first two cases, clinical symptoms were mild, besides in the second case ectopic acromegaly was accompanied by primary hyperparathyroidism. In the third case ectopic acromegaly was accompanied by pituitary macroadenoma, and after surgical removal of the lung NET remission of acromegaly was not achieved. In all three cases, lung NETs were detected incidentally on radiologic chest screening for other conditions.