Department of Gastroenterology, Wenzhou Central Hospital, Wenzhou, China.
Z Gastroenterol. 2024 May;62(5):752-758. doi: 10.1055/a-2170-1691. Epub 2023 Oct 5.
Non-islet cell tumor hypoglycemia (NICTH) is a paraneoplastic syndrome caused by tumors other than insulinoma that is primarily due to excessive production of insulin-like growth factor-II (IGF-II). The prevalence of NICTH is likely underestimated because of a lack of clinical recognition.
A 41-year-old male with massive malignant liver tumors presented with recurrent severe hypoglycemia, weight loss, and liver cirrhosis.
NICTH related to IGF-II produced by hepatocellular carcinoma was diagnosed based on clinical symptoms, biochemical tests, and elevated IGF-II/IGF-I ratio.
Initial treatment with intravenous glucose and parenteral nutrition showed limited efficacy. Glucocorticoids and recombinant human growth hormone led to progressive improvement in blood glucose levels.
Due to extensive tumor burden and liver failure, surgical resection was not feasible, and the patient ultimately succumbed to refractory hypoglycemia and passed away in two weeks.
Early recognition and diagnosis of NICTH are crucial in patients with recurrent hypoglycemia and large tumors. Surgical resection is the preferred treatment option, but supportive care and pharmacological interventions, such as glucocorticoids and growth hormone, can help manage refractory hypoglycemia. Further research is needed to explore novel treatment options, including anti-IGF-I and -IGF-II neutralizing antibodies.
非胰岛细胞瘤性低血糖症(NICTH)是一种由胰岛素瘤以外的肿瘤引起的副肿瘤综合征,主要是由于胰岛素样生长因子-II(IGF-II)的过度产生。由于缺乏临床认识,NICTH 的患病率可能被低估。
一名 41 岁男性,患有巨大恶性肝肿瘤,表现为反复严重低血糖、体重减轻和肝硬化。
根据临床症状、生化检查和 IGF-II/IGF-I 比值升高,诊断为与肝细胞癌产生的 IGF-II 相关的 NICTH。
初始治疗采用静脉葡萄糖和肠外营养,但效果有限。糖皮质激素和重组人生长激素导致血糖水平逐渐改善。
由于肿瘤负荷广泛和肝功能衰竭,手术切除不可行,患者最终死于难治性低血糖,两周内死亡。
对于反复发生低血糖和大肿瘤的患者,早期识别和诊断 NICTH 至关重要。手术切除是首选治疗方法,但支持性护理和药物干预,如糖皮质激素和生长激素,可有助于控制难治性低血糖。需要进一步研究探索新的治疗方法,包括抗 IGF-I 和 IGF-II 中和抗体。