一名患有肺神经内分泌肿瘤患者的肢端肥大症:病例报告及当前文献综述

Acromegaly in a patient with a pulmonary neuroendocrine tumor: case report and review of current literature.

作者信息

Krug Sebastian, Boch Michael, Rexin Peter, Pfestroff Andreas, Gress Thomas, Michl Patrick, Rinke Anja

机构信息

Department of Gastroenterology, Endocrinology and Metabolism, Philipps-University Marburg, Marburg, Germany.

Department of Gastroenterology and Hepatology, Martin-Luther University Halle/Wittenberg, Ernst-Grube Straße 40, 06120, Halle (Saale), Germany.

出版信息

BMC Res Notes. 2016 Jun 27;9:326. doi: 10.1186/s13104-016-2132-1.

Abstract

BACKGROUND

Pulmonary neuroendocrine tumors (NET) form a heterogeneous group of rare diseases. In these tumors, paraneoplastic syndromes have been described to drive the course of the disease, among them acromegaly induced by paraneoplastic secretion of growth hormone-releasing hormone (GHRH).

CASE PRESENTATION

We report the case of a 43 years old patient initially diagnosed with acromegaly accompanied by weight gain and acral enlargement. Subsequently, further diagnostic work-up identified a solitary pulmonary neuroendocrine tumor (NET). Laboratory tests revealed markedly increased growth hormone (GH) and insulin-like growth factor 1 (IGF-1) without GHRH elevation in the absence of pituitary pathologies confirming the paraneoplastic origin of clinical presentation with acromegaly. Curative surgery was performed leading to normalization of the elevated hormone levels and improvement of the clinical symptoms. Immunohistochemically, a typical carcinoid (TC) was seen with low proliferation index and abundant IGF-1 expression.

CONCLUSIONS

The association of acromegaly and pulmonary NET has only rarely been reported. We present an individual case of paraneoplastic GH- and IGF-1 secretion in a patient with pulmonary NET. Based on their rarity, the knowledge of paraneoplastic syndromes occurring in patients with pulmonary NET such as acromegaly due to paraneoplastic GH- and IGF-1 secretion is mandatory to adequately diagnose and treat these patients.

摘要

背景

肺神经内分泌肿瘤(NET)是一组异质性罕见疾病。在这些肿瘤中,已描述副肿瘤综合征可推动疾病进程,其中包括由副肿瘤性生长激素释放激素(GHRH)分泌引起的肢端肥大症。

病例报告

我们报告了一例43岁患者,最初诊断为肢端肥大症,伴有体重增加和肢端增大。随后,进一步的诊断检查发现了一个孤立性肺神经内分泌肿瘤(NET)。实验室检查显示生长激素(GH)和胰岛素样生长因子1(IGF-1)明显升高,而在无垂体病变的情况下GHRH未升高,证实了肢端肥大症临床表现的副肿瘤起源。进行了根治性手术,导致升高的激素水平恢复正常,临床症状改善。免疫组化显示为典型类癌(TC),增殖指数低,IGF-1表达丰富。

结论

肢端肥大症与肺NET的关联仅有很少报道。我们展示了一例肺NET患者副肿瘤性GH和IGF-1分泌的个体病例。鉴于其罕见性,了解肺NET患者中发生的副肿瘤综合征,如因副肿瘤性GH和IGF-1分泌导致的肢端肥大症,对于充分诊断和治疗这些患者至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad58/4924317/2bb0d2e5387d/13104_2016_2132_Fig1_HTML.jpg

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