de Groot Jan Willem B, Rikhof Bart, van Doorn Jaap, Bilo Henk J G, Alleman Maarten A, Honkoop Aafke H, van der Graaf Winette T A
Department of Internal Medicine, Isala Klinieken, Dr van Heesweg 2, 8025 AB Zwolle, The Netherlands.
Endocr Relat Cancer. 2007 Dec;14(4):979-93. doi: 10.1677/ERC-07-0161.
This review focuses on the tumour types and symptoms associated with non-islet cell tumour-induced hypoglycaemia (NICTH) as well as the pathogenesis, diagnosis and treatment of this rare paraneoplastic phenomenon. In addition, we report two illustrative cases of patients suffering from NICTH caused by a solid fibrous tumour and a haemangiopericytoma respectively. In the first case, NICTH resolved following complete resection of the tumour, but in the second case the patient needed long-term treatment aimed at controlling hypoglycaemia because of non-resectable metastases. Many tumour types have been associated with NICTH. The crucial event in the development of NICTH seems to be overexpression of the IGF-II gene by the tumour. NICTH is characterised by recurrent fasting hypoglycaemia and is associated with the secretion of incompletely processed precursors of IGF-II ('big'-IGF-II) by the tumour. This induces dramatic secondary changes in the circulating levels of insulin, GH, IGF-I and IGF-binding proteins, resulting in an insulin-like hypoglycaemic activity of 'big'-IGF-II.
本综述聚焦于与非胰岛细胞瘤所致低血糖症(NICTH)相关的肿瘤类型及症状,以及这种罕见的副肿瘤现象的发病机制、诊断和治疗。此外,我们报告了两例分别由实性纤维瘤和血管外皮细胞瘤引起的NICTH患者的典型病例。在第一例中,肿瘤完全切除后NICTH得以缓解,但在第二例中,由于存在不可切除的转移灶,患者需要长期治疗以控制低血糖。许多肿瘤类型都与NICTH有关。NICTH发生发展的关键事件似乎是肿瘤中IGF-II基因的过表达。NICTH的特征是反复出现空腹低血糖,并与肿瘤分泌未完全加工的IGF-II前体(“大”-IGF-II)有关。这会导致循环中胰岛素、生长激素、IGF-I和IGF结合蛋白水平发生显著的继发性变化,从而产生“大”-IGF-II的胰岛素样降血糖活性。