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[类癌综合征——诊断与管理]

[Carcinoid syndroma - diagnosis and management].

作者信息

Kiňová Soňa, Koreň Michal

出版信息

Vnitr Lek. 2021 Winter;67(5):310-314.

Abstract

Neuroendocrine tumors (NETs, originally termed “carcinoids”) create a relatively rare group of neoplasms with an approximate incidence rate of 5 to 8 cases per 10 000 persons. NETs predominantly demonstrate indolent disease biology for many years. They become symptomatic when they are large enough or when they metastasize to the liver or the lungs, bones, or other sites. Roughly 30% to 40% of subjects with NETs develop carcinoid syndrome. Signs and symptoms of carcinoid syndrome are bronchospasm, flushing, diarrhea and cramping, cyanosis and pellagra. White plaque-like deposits on the endocardial surface of heart structures are characteristic for carcinoid heart disease. The treatment of patients with carcinoid syndrome is multi-faceted due to the necessity to manage simultaneously the systemic cancer disease as well as the signs of carcinoid syndrome and includes resection or debulking of tumor mass, biological treatment with somatostatin analogues and peptide receptor radionuclide treatment.

摘要

神经内分泌肿瘤(NETs,最初称为“类癌”)是一组相对罕见的肿瘤,发病率约为每10000人中有5至8例。多年来,NETs主要表现为惰性疾病生物学行为。当肿瘤足够大或转移至肝脏、肺、骨骼或其他部位时,就会出现症状。大约30%至40%的NETs患者会出现类癌综合征。类癌综合征的体征和症状包括支气管痉挛、潮红、腹泻和绞痛、发绀和糙皮病。心脏结构心内膜表面的白色斑块样沉积物是类癌心脏病的特征。由于需要同时治疗全身性癌症疾病以及类癌综合征的体征,类癌综合征患者的治疗是多方面的,包括切除或减少肿瘤肿块、用生长抑素类似物进行生物治疗和肽受体放射性核素治疗。

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