Unlabelled: We describe the clinical presentation, diagnostic and management issues in five cases of non-islet cell tumor hypoglycemia (NICTH), diagnosed at a tertiary care institute over a period of 15 years. The clinical, laboratory, and histopathological findings of these patients along with diagnostic utility of IGF2:IGF1 ratio are discussed. The mean age of presentation was 52 years, with a male predominance (3:2). Three patients presented with recurrent episodes of fasting hypoglycemia and it was detected in other two patients during hospitalization. Two patients had acromegaloid features that regressed following treatment. One patient had hypokalemia. Low levels of insulin, C-peptide, GH, and IGF1 were invariably found in all. The IGF2 level was elevated in only one patient; however, IGF2:IGF1 ratio was more than 10 in four of the five patients. The mean tumor size was 16.4 cm and mean weight was 3.6 kg. Four patients had mesenchymal tumors and one had epithelial tumor. NICTH is a rare cause of hypoglycemia. Hypoinsulinemic hypoglycemia with low IGF1 and IGF2:IGF1 ratio more than 10 is suggestive of this entity.
Learning Points: NICTH should be considered in patients presenting with tumor of mesenchymal origin and hypoglycemia.Hypoinsulinemic hypoglycemia with low IGF1 is a strong biochemical evidence of NICTH.IGF2:IGF1 ratio of more than 10 is a complementary investigation in the absence of an assay facility for IGF2.
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http://dx.doi.org/10.1530/EDM-13-0046 | DOI Listing |
Cureus
July 2024
Endocrinology, Faculty of Medicine, Fattouma Bourguiba University Hospital of Monastir, Monastir, TUN.
Non-islet cell tumor hypoglycemia (NICTH) is a rare paraneoplastic syndrome characterized by insulin-like growth factor-2 (IGF-2) release, often associated with diverse tumor types. Gastrointestinal stromal tumors (GISTs), sarcomatous lesions of the gastrointestinal tract, are rarely associated with NICTH. We present a unique case of a 58-year-old patient diagnosed with a GIST exhibiting recurrent hypoglycemia suggestive of NICTH.
View Article and Find Full Text PDFEndocrinol Diabetes Metab Case Rep
January 2024
Division of Endocrinology & Metabolism, Faculty of Medicine & Dentistry, University of Alberta, Edmonton, Alberta, Canada.
Summary: Non-islet cell tumour hypoglycemia (NICTH), typically mediated by insulin-like growth factor 2 (IGF-2), is a rare but highly morbid paraneoplastic syndrome associated with tumours of mesenchymal or epithelial origin. Outside of dextrose administration and dietary modification which provide transient relief of hypoglycemia, resection of the underlying tumour is the only known cure for NICTH. Available medical therapies to manage hypoglycemia include glucocorticoids, recombinant growth hormone, and pasireotide.
View Article and Find Full Text PDFJ Clin Med
June 2023
Endocrinology Unit, Department of Medical and Surgical Sciences, University of Foggia, 71122 Foggia, Italy.
Hypoglycemia in patients without diabetes is a diagnostic challenge for the endocrinologist. Sometimes it is related to rare causes such as Doege-Potter Syndrome (DPS). DPS is caused by an abnormal insulin-like grow factor 2(IGF-2) that retains part of the E domain during the production process, resulting in a longer peptide called "big-IGF-2".
View Article and Find Full Text PDFActa Endocrinol (Buchar)
January 2022
"Victor Babes" University of Medicine and Pharmacy, Internal Medicine - Diabetes, Nutrition, Metabolic Diseases, and Systemic Rheumatology, Timisoara, Romania.
Hypoglycemia from a non-insulin-secreting extrapancreatic tumor is a paraneoplastic syndrome characterized by the tumor production of a substance with an insulin-like effect (insulin-like growth factor 1 or 2 - IGF 1 or 2). Diagnosis is confirmed by the determination of IGF-1 and IGF-2 and finding an elevated IGF - 2/IGF-1 ratio. The availability of these tests is limited in many laboratories, so prompt recognition and therapies are late.
View Article and Find Full Text PDFFront Endocrinol (Lausanne)
May 2019
Department of Internal Medicine, University of Mississippi Medical Center, Jackson, MS, United States.
Non-islet cell hypoglycemia (NICH) is hypoglycemia due to the overproduction of insulin-like growth factor-2 (IGF-2) and its precursors which can activate the insulin receptor. Typically, large mesenchymal and epithelial tumors can cause NICH. Diagnosis is confirmed by finding an elevated IGF-2/IGF-1 ratio.
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