Department of endocrinology and metabolism, CHRU Lille, 59037 Lille, France.
Department of medicine and endocrinology, general hospital, 62100 Calais, France.
Ann Endocrinol (Paris). 2019 Feb;80(1):21-25. doi: 10.1016/j.ando.2018.01.005. Epub 2018 Mar 16.
The purpose of this study was to analyse the characteristics of 6 patients managed in a university hospital between 1996 and 2016 for non-islet cell tumor hypoglycemia (NICTH), a form of hypoglycaemia due to the paraneoplastic secretion of IGF-2 or its related substances. RESULTS: Three of these 6 patients (50%), aged over 69 years, including 2 with acromegaloid phenotype, presented with a pleural solitary fibrous tumor (SFT), with median diameter 20 cm (interquartile range, 12.5-20.5) with a low median SUV (3.3 g/mL (QR, 2-7.5)) on 18F-FDG PET. The other 3 patients presented respectively neuroendocrine carcinoma (NEC) of the palate (70-year-old woman), retroperitoneal myxofibrosarcoma (66-year-old man) and meningeal hemangiopericytoma (36-year-old woman). All 3 were inoperable and did not respond to any therapy other than glucose solution. Corticosteroid therapy was effective in the 3 SFTs and the NEC. One of the SFTs recurred 10 years later with asymptomatic hypoglycemia, which resolved after reintervention. Median (IQR) blood glucose levels of the 6 patients was 0.4g/L (QR, 0.31-0.41), with hypoinsulinemia at 0.7mIU/L (QR 0.7-2.0), undetectable GH, low IGF-1, normal IGF-2 level in 5/6 cases, a high IGF-2:IGF-1 ratio at 26.9 (QR, 20.8-37.8), hypokalemia and hypomagnesemia. CONCLUSION: NICTH is a rare syndrome, which should be considered in the presence of hypoinsulinemic hypoglycemia with low GH and IGF-1, and a IGF-2:IGF-1 ratio>10. Corticosteroid therapy was effective in elderly subjects, particularly with solitary fibrous tumor, which was generally operable. Hemangiopericytoma and myxofibrosarcoma had poor prognosis in younger patients.
这项研究的目的是分析 1996 年至 2016 年间在一所大学医院接受治疗的 6 例非胰岛细胞瘤性低血糖症（NICTH）患者的特征，NICTH 是一种由于 IGF-2 或其相关物质的副肿瘤分泌而导致的低血糖症形式。结果：这 6 例患者中有 3 例（50%）年龄超过 69 岁，其中 2 例有肢端肥大症表型，表现为胸膜孤立性纤维瘤（SFT），平均直径 20cm（四分位距，12.5-20.5cm），平均 SUV（3.3g/ml（四分位距，2-7.5））在 18F-FDG PET 上。另外 3 例患者分别为腭部神经内分泌癌（NEC）（70 岁女性）、腹膜后粘液纤维肉瘤（66 岁男性）和脑膜血管外皮细胞瘤（36 岁女性）。所有 3 例患者均无法手术，除葡萄糖溶液外，其他治疗均无效。皮质类固醇治疗对 3 例 SFT 和 NEC 有效。其中 1 例 SFT10 年后复发，伴无症状性低血糖，再次干预后缓解。6 例患者的中位（IQR）血糖水平为 0.4g/L（IQR，0.31-0.41），低胰岛素血症为 0.7mIU/L（IQR，0.7-2.0），不可检测的 GH、低 IGF-1，5/6 例患者的 IGF-2 水平正常，IGF-2:IGF-1 比值为 26.9（IQR，20.8-37.8），低钾血症和低镁血症。结论：NICTH 是一种罕见的综合征，当存在低血糖伴低 GH 和 IGF-1，IGF-2:IGF-1 比值>10 时，应考虑 NICTH。皮质类固醇治疗对老年患者，特别是孤立性纤维瘤患者有效，孤立性纤维瘤通常可手术切除。血管外皮细胞瘤和粘液纤维肉瘤在年轻患者中的预后较差。
