Tumours producing hypoglycaemia

Teale, J D

doi:10.1677/erc.0.0050111

Cited by 80 publications

(57 citation statements)

References 147 publications

(129 reference statements)

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“…Thus, the IGF-2: IGF-1 ratio is used as a surrogate marker for big IGF-2 concentration. A ratio of >10 is considered to be clinically significant [42]. …”

Section: Discussionmentioning

confidence: 99%

Hypoglycemia mediated by paraneoplastic production of Insulin like growth factor–2 from a malignant renal solitary fibrous tumor – clinical case and literature review

et al. 2014

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BackgroundHypoglycemic episodes are infrequent in individuals without a history of diabetes mellitus or bariatric surgery. When hypoglycemia does occur in such individuals, an uncommon but important diagnosis to consider is non-islet cell tumor hypoglycemia (NICTH). We report a case of NICTH associated with paraneoplastic insulin-like growth factor-2 (IGF-2) production and review current relevant medical literature.Case presentationA 60 year old male with no relevant past medical history was referred to the endocrinology clinic with 18 month history of episodic hypoglycemic symptoms and, on one occasion was noted to have a fingerstick glucose of 36 mg/dL while having symptoms of hypoglycemia. Basic laboratory evaluation was unrevealing. Further evaluation however showed an elevated serum IGF-2 level at 2215 ng/mL (reference range 411–1248 ng/mL). Imaging demonstrated a large right suprarenal mass. A right nephrectomy with resection of the mass demonstrated a malignant solitary fibrous tumor. Post resection, the patient’s IGF-2 levels normalized and hypoglycemic symptoms resolved.ConclusionDue to the structural and biochemical homology between IGF-2 and insulin, elevated levels of IGF-2 can result in hypoglycemia. A posttranslational precursor to IGF-2 known as “big IGF” also possesses biologic activity. Review of recent reported cases of NICTH identified widespread anatomic locations and varied pathologic diagnoses of tumors associated with paraneoplastic production of IGF-2 causing hypoglycemia. Definitive management of hypoglycemia associated with paraneoplastic production of IGF-2 consists of resection of the tumor responsible for IGF-2 production. Accumulating literature provides a firm basis for routine IGF-2 laboratory evaluation in patients presenting with spontaneous hypoglycemia with no readily apparent cause.

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“…Thus, the IGF-2: IGF-1 ratio is used as a surrogate marker for big IGF-2 concentration. A ratio of >10 is considered to be clinically significant [42]. …”

Section: Discussionmentioning

confidence: 99%

Hypoglycemia mediated by paraneoplastic production of Insulin like growth factor–2 from a malignant renal solitary fibrous tumor – clinical case and literature review

et al. 2014

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“…Therapeutic options for NICTH include complete removal of the tumor or debulking followed by radiotherapy and chemotherapy for inoperable disease. Glucocorticoid therapy is the most effective medical treatment for long-term relief (8) (9) (10). It ameliorates hypoglycemia by increasing gluconeogenesis and by suppressing the production of ‘big’ IGF2.…”

Section: Discussionmentioning

confidence: 99%

Non-islet cell tumor-induced hypoglycemia: a report of five cases and brief review of the literature

Dutta

Aggarwal

Gogate

et al. 2013

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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SummaryWe 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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“…Although the exact incidence of NICTH is unknown, it is estimated to be about 1/4th of the incidence of insulinoma, but is probably underestimated because of occult disease (1). …”

Section: Introductionmentioning

confidence: 99%

Pro-IGF2-induced hypoglycaemia associated with hepatocellular carcinoma

Berg

2017

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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We present a patient (87 years, female) who was admitted to the emergency department because of loss of consciousness. Previous medical history included advanced-stage hepatocellular carcinoma and associated weight loss. She was found on the ground in an unresponsive state by her daughter and was determined to be hypoglycaemic. Upon bolus administration of 100 mL intravenous glucose (10%), glucose levels increased to 2.9 mmol/L and the patient regained full consciousness. She was admitted to the hospital for further examination, and treatment and continuous intravenous glucose infusion was initiated. As the patient was known to suffer from advanced-stage hepatocellular carcinoma, tumour-associated hypoglycaemia was suspected. Insulin, c-peptide and IGF1 concentrations were indeed low, cortisol concentration was high and IGF2 and Pro-IGF2 were borderline low and borderline high normal respectively. IGF2:IGF1 ratio was 23, confirming the diagnosis of non-islet cell tumour hypoglycaemia. During the initial phase of treatment, euglycaemia was maintained by continuous variable glucose infusion (5%, varying between 1 and 2 L/24 h), and the patient was advised to eat small snacks throughout the day. After euglycaemia was established and the diagnosis was confirmed, prednisolone was started (30 mg, 1 dd) and glucose infusions were halted. Under prednisolone treatment, glucose levels were slightly increased and no further hypoglycaemic episodes occurred. At her request, no surgery was performed. After 19 days, the patient was discharged to a hospice and died 3 weeks later.Learning points:Hepatocellular carcinoma may be associated with non-islet cell tumour hypoglycaemia (NICTH).NICTH-induced hypoglycaemia is associated with low insulin and IGF1.Measurement of IGF2 only (without measurement of Pro-IGF2 and IGF1) may be insufficient to prove NICTH.

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Tumours producing hypoglycaemia

Cited by 80 publications

References 147 publications

Hypoglycemia mediated by paraneoplastic production of Insulin like growth factor–2 from a malignant renal solitary fibrous tumor – clinical case and literature review

Hypoglycemia mediated by paraneoplastic production of Insulin like growth factor–2 from a malignant renal solitary fibrous tumor – clinical case and literature review

Non-islet cell tumor-induced hypoglycemia: a report of five cases and brief review of the literature

Pro-IGF2-induced hypoglycaemia associated with hepatocellular carcinoma

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