Searchable abstracts of presentations at key conferences in endocrinology
Endocrine Abstracts (2024) 100 OC2 | DOI: 10.1530/endoabs.100.OC2

SFEEU2024 Society for Endocrinology National Clinical Cases 2024 Oral Communications (10 abstracts)

Non-islet cell tumour hypoglycaemia in adrenocortical carcinoma responding to combined growth hormone and corticosteroid therapy

C E Plowman 1 , O Soruna 1 , J Pittaway 1 , E Goodchild 1 , J Shamash 2 , TT Chung 3 , T E Abdel-Aziz 3 & W M Drake 1

1Department of Endocrinology, St Bartholomew’s Hospital, London, EC1A 7BE, United Kingdom; 2Department of Clinical Oncology, London, EC1A 7BE, United Kingdom; 3Department of Endocrinology, University College London Hospital, London, NW1 2BU, United Kingdom

Case History: A 26-year-old woman with known metastatic adrenocortical carcinoma (ACC) was transferred to our department with recurrent severe hypoglycaemic episodes. Her ACC was diagnosed in 2018 and a 175 × 130 × 89 mm 1200 g mass was surgically resected (ENS@T stage-II, Weiss score-5, Ki-67 20%, Helsinki score-28). Her disease progressed despite adjuvant radiotherapy, mitotane and three chemotherapy regimens. In January 2023, she began experiencing episodes of profound hypoglycaemia, associated with hypokalaemia. She had loss of hypoglycaemic awareness and had prolonged hospitalisations for collapse and seizures.

Investigations and method: Her diagnostic work up centred on serum samples taken during a hypoglycaemic episode, including c-peptide, insulin, growth hormone (GH), insulin-like growth factor (IGF)-1 and sulphonylurea screen. Subsequent IGF-I and IGF-II assays were done before and after initiating treatment.

Results and treatment: The raised IGF-I:IGF-II ratio (30.3, normal <10) confirmed non-islet cell tumour hypoglycaemia (NICTH). A trial of GH (somatropin 1.2 mg OD subcutaneously) and corticosteroids (dexamethasone 1 mg OD) was started. Her hypoglycaemia and hypokalaemia resolved within days and her IGF-I:IGF-II ratio improved. Unfortunately, her ACC progressed and she died in February 2024.

Results and treatment
Fasting plasma glucose (3.5-6 mmol/l) 1.4
Potassium(3.5-5.3 mmol/l)2.4
C-peptide(370-1470 pmol/l)<50
Insulin(2.6-24.9 mU/l)<1.0
Urine sulphonylurea screennegative
Growth Hormone (mg/l)0.60
IGF-1 (103.3-328.4 mg/l)39
Table 1.
Test February-23April-23May-23November-23
IGF-I(11.2-54.5 nmol/l) 2.3GH and corticosteroids started11.311.2
IGF-II(nmol/l) 69.7139.485.5
IGF-II:IGF-I ratio(<10)30.312.37.6

Conclusions: ACCs are rare; although 50-60% are functional they typically produce glucocorticoids and/or mineralocorticoids and/or or androgens. Although many ACCs are associated with overexpression of IGF-II, aberrant expression of the immature form of IGF-II believed to be important in the mechanism of NICTH is very rare. NICTH can be suspected in the context of malignancy with recurrent hypoglycaemia, exclusion of the presence of endogenous/exogenous insulin and is supported by a raised serum IGF-I:IGF-II ratio. After initial stabilisation with glucagon and glucose, management can be surgical or with medical therapy. Treatment options include GH and/or corticosteroids. Somatostatin analogues have been used with varying effect. This is a very rare case of metastatic ACC secreting pro-IGF-II causing NICTH. The hypoglycaemic episodes responded well to combination treatment with corticosteroids and GH. The use of GH in this setting is a risk-benefit decision; the theoretical mitogenic effects of GH on the ACC were offset by the need to treat life-threatening hypoglycaemia.

Article tools

My recent searches

No recent searches.