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2015 ; 2015
(ä): 150062
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Severe paraneoplastic hypoglycemia secondary to a gastrointestinal stromal tumour
masquerading as a stroke
#MMPMID26535131
Dimitriadis GK
; Gopalakrishnan K
; Rao R
; Grammatopoulos DK
; Randeva HS
; Weickert MO
; Murthy N
Endocrinol Diabetes Metab Case Rep
2015[]; 2015
(ä): 150062
PMID26535131
show ga
We report the case of a 70-year-old previously healthy female who presented
acutely to the Accident and Emergency department with left-sided vasomotor
symptoms including reduced muscle tone, weakness upon walking and slurred speech.
Physical examination confirmed hemiparesis with VIIth nerve palsy and profound
hepatomegaly. A random glucose was low at 1.7?mmol/l, which upon correction
resolved her symptoms. In hindsight, the patient recalled having had similar
episodes periodically over the past 3 months to which she did not give much
attention. While hospitalized, she continued having 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 Synacthen stimulation test was normal (Cort
(0') 390?nmol/l, Cort (30') 773?nmol/l). A computed tomography scan showed
multiple lobulated 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 further biochemical investigations
(IGF2=96.5?nmol/l; IGF2:IGF1 ratio 18.9, ULN <10). Treatment with growth hormone
resolved the patient's hypoglycaemic symptoms and subsequent targeted therapy
with Imatinib was successful in controlling disease progression over an 8-year
observation period. LEARNING POINTS: NICTH can be a rare complication of GISTs
that may manifest with severe hypoglycaemia and neuroglucopenic symptoms.NICTH
can masquerade as other pathologies thus causing diagnostic
confusion.Histological confirmation of GIST induced NICTH and exclusion of other
conditions causing hypoglycaemia is essential.Mutational analysis of GISTs should
be carried out in all cases as it guides treatment decision.Tailored management
of hypoglycaemia, in this case using growth hormone and targeted cyto-reductive
therapy, minimizes the risk of possible life-threatening complications.