Reisch N, Reincke M
Medizinische Klinik IV, Klinikum der Universität München, Ziemssenstr. 1, 80336, München, Deutschland.
Internist (Berl). 2018 Feb;59(2):125-133. doi: 10.1007/s00108-017-0377-y.
Endocrine paraneoplastic syndromes result from the production of bioactive substances from neoplastic cells, of endocrine or neuroendocrine origin. Typically these are located in the lungs, the gastrointestinal tract, pancreas, thyroid gland, adrenal medulla, skin, prostate or breast. In endocrine paraneoplastic syndromes the secretion of peptides, amines or other bioactive substances is always ectopic and not related to the anatomical source. The clinical presentation, however, is indistinguishable from a suspected eutopic endocrine tumor posing a diagnostic challenge. The most common endocrine paraneoplastic syndromes are based on the secretion of antidiuretic hormone (ADH) resulting in hyponatremia, secretion of adrenocorticotropic hormone (ACTH) or rarely corticotropin-releasing hormone (CRH) resulting in Cushing syndrome as well as secretion of growth hormone-releasing hormone resulting in acromegaly. Paraneoplastic endocrine syndromes mainly occur in highly malignant tumors; however, the development of these tumors does not necessarily correlate with tumor stage, malignant potential or prognosis. As endocrine paraneoplastic syndromes are a rare complication, there are hardly any evidence-based therapeutic recommendations. Treatment of the underlying tumor is the first choice and in a palliative setting symptomatic therapy is possible.
内分泌副肿瘤综合征是由肿瘤细胞产生具有生物活性的物质所致,这些肿瘤细胞起源于内分泌或神经内分泌组织。通常这些肿瘤位于肺、胃肠道、胰腺、甲状腺、肾上腺髓质、皮肤、前列腺或乳腺。在内分泌副肿瘤综合征中,肽类、胺类或其他生物活性物质的分泌总是异位的,与解剖学来源无关。然而,其临床表现与疑似原位内分泌肿瘤难以区分,这给诊断带来了挑战。最常见的内分泌副肿瘤综合征是由于抗利尿激素(ADH)分泌导致低钠血症,促肾上腺皮质激素(ACTH)分泌或罕见的促肾上腺皮质激素释放激素(CRH)分泌导致库欣综合征,以及生长激素释放激素分泌导致肢端肥大症。副肿瘤内分泌综合征主要发生于高度恶性肿瘤;然而,这些肿瘤的发生并不一定与肿瘤分期、恶性潜能或预后相关。由于内分泌副肿瘤综合征是一种罕见的并发症,几乎没有基于证据的治疗建议。治疗基础肿瘤是首选,在姑息治疗中,对症治疗也是可行的。