Refractory hypoglycemia in a patient with functional adrenal cortical carcinoma. (4th November 2016)

• Record Type: Journal Article
• Authors: Marchetti, Katia Regina; Pereira, Maria Adelaide Albergaria; Lichtenstein, Arnaldo; Paiva, Edison Ferreira
• Is Part Of: Endocrinology, diabetes & metabolism case reports. (2016)
• Publication Date: 2016-11-04
• Subjects: Adult -- Female -- Black - other -- Brazil; Adrenal -- Adrenal -- GH -- IGF1 -- IGF2 -- Androstenedione -- Dehydroepiandrostenedione -- ACTH -- Testosterone -- Insulin -- Adrenocortical carcinoma -- Cushing's syndrome -- Hypoglycaemia -- Hyperandrogenism -- Hypokalaemia -- Hypoinsulinaemia -- Non-islet-cell tumour hypoglycaemia; Hypoglycaemia -- Confusion -- Vision – blurred -- Hypercortisolaemia -- Oedema -- Dyspnoea -- Hyperandrogenism -- Acne -- Bloating -- Nausea -- Hypoinsulinaemia -- Hypokalaemia -- Weight loss -- Appetite reduction/loss -- Myasthaenia -- Liver masses -- Hypertension -- Xeroderma -- Acanthosis nigricans -- Metabolic alkalosis -- Facies – abnormal -- Hirsutism -- C-peptide (blood) -- GH -- IGF1 -- IGF2 -- CT scan -- Potassium -- ACTH -- Albumin -- Insulin -- Bicarbonate -- Cortisol (serum) -- Cortisol (salivary) -- Cortisol, free (24-hour urine) -- Androstenedione -- Dehydroepiandrostenedione -- Liver biopsy -- Glucose suppression test -- Immunohistochemistry -- Testosterone -- Chemotherapy -- Glucagon -- Somatostatin analogues -- Potassium chloride -- Octreotide -- Glucose -- Cisplatin -- Amlodipine -- Doxorubicin -- Anthracyclines -- Mitotane -- Etoposide -- Spironolactone; General practice -- Oncology -- Radiology/Rheumatology; Insight into disease pathogenesis or mechanism of therapy -- November -- 2016; Endocrinology -- Periodicals; Diabetes -- Periodicals; Diabetes Mellitus; Endocrinology; Diabetes; Endocrinology; Case Reports; Periodicals; Periodicals; 616.4
• DOI: 10.1530/EDM-16-0101
• Languages: English
• ISSNs: 2052-0573

Abstract

Summary: Adrenacarcinomas are rare, and hypoglycemic syndrome resulting from the secretion of insulin-like growth factor II (IGF-II) by these tumors have been described infrequently. This study describes the case of a young woman with severe persistent hypoglycemia and a large adrenal tumor and discusses the physiopathological mechanisms involved in hypoglycemia. The case is described as a 21-year-old woman who presented with 8 months of general symptoms and, in the preceding 3 months, with episodes of mental confusion and visual blurring secondary to hypoglycemia. A functional assessment of the adrenal cortex revealed ACTH-independent hypercortisolism and hyperandrogenism. Hypoglycemia, hypoinsulinemia, low C-peptide and no ketones were also detected. An evaluation of the GH–IGF axis revealed GH blockade (0.03; reference: up to 4.4 ng/mL), greatly reduced IGF-I levels (9.0 ng/mL; reference: 180–780 ng/mL), slightly reduced IGF-II levels (197 ng/mL; reference: 267–616 ng/mL) and an elevated IGF-II/IGF-I ratio (21.9; reference: ~3). CT scan revealed a large expansive mass in the right adrenal gland and pulmonary and liver metastases. During hospitalization, the patient experienced frequent difficult-to-control hypoglycemia and hypokalemia episodes. Octreotide was ineffective in controlling hypoglycemia. Due to unresectability, chemotherapy was tried, but after 3 months, the patient's condition worsened and progressed to death. In conclusion, our patient presented with a functional adrenal cortical carcinoma, with hyperandrogenism associated with hypoinsulinemic hypoglycemia and blockage of the GH–IGF-I axis. Patient's data suggested a diagnosis of hypoglycemia induced by an IGF-II or a large IGF-II-producing tumor (low levels of GH, greatly decreased IGF-I, slightly decreased IGF-II and an elevated IGF-II/IGF-I ratio). Learning points: Hypoglycemyndrome resulting from the secretion of insulin-like growth factor II (IGF-II) by adrenal tumors is a rare condition. Hypoinsulinemic hypoglycemia associated with hyperandrogenism and blockage of the GH–IGF-I axis suggests hypoglycemia induced by an IGF-II or a large IGF-II-producing tumor. Hypoglycemia in cases of NICTH should be treated with glucocorticoids, glucagon, somatostatin analogs and hGH.