Haeri Nami Safai, Mahmud Hussain, Korytkowski Mary T
Division of Endocrinology and Metabolism, Department of Medicine, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania.
AACE Clin Case Rep. 2021 May 28;7(6):376-378. doi: 10.1016/j.aace.2021.05.006. eCollection 2021 Nov-Dec.
Non-islet cell tumor hypoglycemia (NICTH) is an uncommon paraneoplastic syndrome associated with mesenchymal neoplasms such as gastrointestinal stromal tumors (GISTs). We report the case of a patient with type 1 diabetes (T1D) and recurrent GIST who not only required discontinuation of insulin therapy but also required continuous parenteral glucose infusions to prevent hypoglycemia.
A 59-year-old woman with a 24-year history of T1D and recurrent GIST presented with frequent episodes of symptomatic hypoglycemia despite continuous reductions in her insulin therapy. Laboratory workup revealed undetectable insulin and C-peptide, low insulin-like growth factor (IGF) 1, normal IGF-2, and an elevated IGF-2:IGF-1 ratio. Medical management with prednisone alone and, later, in combination with octreotide did not reduce hypoglycemic episodes. Eventually, during hospitalization for severe hypoglycemia, she was treated and discharged with continuous intravenous dextrose infusion. She ultimately required around-the-clock glucose infusions, which helped her maintain what she believed was an acceptable quality of life during her remaining weeks.
NICTH is characterized by excessive tumor production of IGF-2 or pro-IGF-2, leading to unrestricted glucose uptake in peripheral tissues and hypoglycemia. A diagnosis of NICTH can be made on the basis of low IGF-1 levels in the plasma with normal or elevated IGF-2. Tumor resection is the most definitive treatment for NICTH.
This patient with T1D presented with resistant hypoglycemia due to recurrence of an enlarging GIST. She required discontinuation of all insulin therapy and continuous dextrose infusions to maintain euglycemia.
非胰岛细胞瘤低血糖症（NICTH）是一种罕见的副肿瘤综合征，与间质肿瘤如胃肠道间质瘤（GIST）相关。我们报告一例1型糖尿病（T1D）合并复发性GIST患者的病例，该患者不仅需要停用胰岛素治疗，还需要持续胃肠外输注葡萄糖以预防低血糖。
一名有24年T1D病史且GIST复发的59岁女性，尽管胰岛素治疗持续减少，但仍频繁出现症状性低血糖发作。实验室检查显示胰岛素和C肽检测不到、胰岛素样生长因子（IGF）1水平低、IGF-2正常以及IGF-2:IGF-1比值升高。单独使用泼尼松以及后来联合奥曲肽进行药物治疗并未减少低血糖发作次数。最终，在因严重低血糖住院期间，她接受了持续静脉输注葡萄糖治疗并出院。她最终需要全天候葡萄糖输注，这帮助她在剩余的几周里维持了她认为可接受的生活质量。
NICTH的特征是肿瘤过度产生IGF-2或前IGF-2，导致外周组织不受限制地摄取葡萄糖并引发低血糖。基于血浆中IGF-1水平低而IGF-2正常或升高可诊断NICTH。肿瘤切除是NICTH最确切的治疗方法。
该T1D患者因增大的GIST复发出现难治性低血糖。她需要停用所有胰岛素治疗并持续输注葡萄糖以维持血糖正常。
