Severe paraneoplastic hypoglycemia secondary to a gastrointestinal stromal tumour masquerading as a stroke

[摘要] We report the case of a 70-year-old previously healthy female who presented acutely to the Accident and Emergencydepartment with left-sided vasomotor symptoms including reduced muscle tone, weakness upon walking and slurredspeech. Physical examination confirmed hemiparesis with VIIth nerve palsy and profound hepatomegaly. A random glucosewas low at 1.7 mmol/l, which upon correction resolved her symptoms. In hindsight, the patient recalled having had similarepisodes periodically over the past 3 months to which she did not give much attention. While hospitalized, she continuedhaving episodes of symptomatic hypoglycaemia during most nights, requiring treatment with i.v. dextrose and/or glucagon.Blood tests including insulin and C-peptide were invariably suppressed, in correlation with low glucose. A Synacthenstimulation test was normal (Cort (00) 390 nmol/l, Cort (300) 773 nmol/l). A computed tomography scan showed multiplelobulated masses in the abdomen, liver and pelvis. An ultrasound guided biopsy of one of the pelvic masses was performed.Immunohistochemistry supported the diagnosis of a gastrointestinal stromal tumour (GIST) positive for CD34 and CD117.A diagnosis of a non islet cell tumour hypoglycaemia (NICTH) secondary to an IGF2 secreting GIST was confirmed with furtherbiochemical investigations (IGF2Z96.5 nmol/l; IGF2:IGF1 ratio 18.9, ULN !10). Treatment with growth hormone resolvedthe patient’s hypoglycaemic symptoms and subsequent targeted therapy with Imatinib was successful in controlling diseaseprogression over an 8-year observation period.

[学科分类] 血液学

[关键词] Geriatric;Female;White;United Kingdom;Duodenum;Stomach;Endocrine-related cancer;IGF2;Insulin;Hypoglycaemia;Non-islet-cell tumour hypoglycaemia;Gastrointestinal stromal tumour;Hypoglycaemia;Headache;Paraesthesia;Ptosis;Pelvic mass;Hemiparesisy;Facial palsy;Hepatomegaly;Myasthaenia;CT scan;Immunohistochemistry;Glucose (blood);Biopsy;Ultrasound-guided biopsy;IGF 2;Insulin;C-peptide (blood);Imatinib;Tyrosine-kinase inhibitors;Glucagon;GH;Prednisolone;Glucocorticoids;Glucose;Gastroenterology;Unique/unexpected symptoms or presentations of a disease;October;2015