A Case of Non-Islet Cell Tumor Hypoglycemia (NICTH) Associated with Gastrointestinal Stromal Tumor (GIST)

Abstract

Patient: Male, 60Final Diagnosis: Non-islet cell tumor hypoglycemia (NICTH) associated with gastrointestinal stromal tumor (GIST)Symptoms: HypoglycemiaMedication: —Clinical Procedure:—Specialty: Gastroenterology and HepatologyObjective:Rare diseaseBackground:Non-islet cell tumor hypoglycemia (NICTH) is a newly recognized, but uncommon, paraneoplastic syndrome that is associated with tumors of mesenchymal origin. We report a case of NICTH associated with a gastrointestinal stromal tumor (GIST).Case Report:A 60-year-old man presented to the emergency department of our hospital after being found unconscious in his home. His serum blood glucose on hospital admission was 40 mg/dL. He reported a three-month history of diffuse abdominal pain, fatigue, and blurred vision. Laboratory medicine investigations showed reduced levels of insulin, C-peptide, insulin-like growth factor binding protein (IGFBP)-3, and insulin-like growth factor (IGF)-1, but his IGFBP-2 was increased. Computed tomography (CT) scan of the chest and abdomen showed an abdominal mass that involved the small bowel, mesentery, and omentum, with lesions in the right lung and the left rib. Histopathology of a CT-guided biopsy of the abdominal mass showed a low-grade sarcomatous spindle cell neoplasm that was positive for CD117 using immunohistochemistry and with an exon 11 c-KIT mutation. These findings were consistent with a diagnosis of GIST and treatment with imatinib commenced.Conclusions:This case report has shown that hypoglycemia in the setting of low levels of insulin, C-peptide, IGF-1, and IGFBP-3 is suggestive of a diagnosis of NICTH, which should be investigated for an underlying source, which in this case, was confirmed to be a malignant GIST.

Electroencephalographic alterations in a mare with presumed intermittent neuroglycopenia caused by severe hypoglycaemia‐associated with nonislet cell tumour

“…'Big IGF-II' is a large molecular weight pro-peptide with potent insulin-like activity. NICTH mediated by excessive secretion of IGF-I has also been reported (Wilson et al 2017).…”

“…; Wilson et al . ). In dogs, NICTH has been reported mostly in hepatic tumours (hepatocellular carcinoma, hepatic leiomyosarcoma and hepatic haemangiosarcoma), as well as splenic haemangiosarcoma, melanoma, lymphoma, multiple myeloma and mammary carcinoma (Leifer et al .…”

“…NICTH mediated by excessive secretion of IGF‐I has also been reported (Wilson et al . ). The mechanism of the hypoglycaemia in the present case of renal (tubular) carcinoma with carcinomatosis was not determined.…”

“…Nonislet cell tumour-induced hypoglycaemia has been reported in horses with primary neoplasia of the kidney (Berggren 1980;Van Mol and Fransen 1986;West et al 1987;Baker et al 2001;Swain et al 2005;Lyle et al 2010), primary neoplasia of the liver (Roby et al 1990;Gold et al 2008;Wong et al 2015), as well as mesothelioma (LaCarrubba et al 2006) and gastrointestinal stromal tumour (Haga et al 2008). NICTH in humans has been associated mostly with neoplasia of mesenchymal or hepatic origin (de Groot et al 2007;Bodnar et al 2014;Khowaja et al 2014;Han et al 2017;Wilson et al 2017). In dogs, NICTH has been reported mostly in hepatic tumours (hepatocellular carcinoma, hepatic leiomyosarcoma and hepatic haemangiosarcoma), as well as splenic haemangiosarcoma, melanoma, lymphoma, multiple myeloma and mammary carcinoma (Leifer et al 1985;Finora 2003;Rossi et al 2010).…”

Novel case of a scleroderma-mimicking syndrome associated with gastrointestinal stromal tumour

“…To our knowledge, this is the first reported case of GISTrelated scleroderma-like syndrome in the literature, making this a unique and valuable case. Rare cases of paraneoplastic syndromes associated with GIST have been reported: hypoglycaemia, [6][7][8][9][10] alopecia areata 11 and hypercalcaemia. 12 Samimi et al 13 described a case of GIST-related dermatomyositis.…”