Persistent hypoglycemia due to an IGF‐II‐secreting malignant pheochromocytoma: a case report and literature review

García, María Martínez; Aznar, Pablo Trincado; Alaminos, María Elena López; Fernandez, M. Gonzalez; Rosas, Almendra Alvarado; Laclaustra, Martín

doi:10.1002/ccr3.3161

Cited by 4 publications

(4 citation statements)

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“…The authors of first report described a fatal case of pheochromocytoma that presented with intractable hypoglycemia with elevated levels of IGF-2 as the most likely cause of hypoglycemia. 20 In the second report, the authors proposed that direct tumor consumption was the cause of hypoglycemia based on F-2-fluorodeoxy-D- glucose positron emission tomography imaging of the tumor. 18 The authors of the third report suggested that direct eutopic islet cell response to the pheochromocytoma with high and inappropriate levels of insulin was the underlying cause of the hypoglycemia.…”

Section: Discussionmentioning

confidence: 99%

Tumor-Induced Hypoglycemia: An Unusual Case Report and Review of Literature

Abdulhadi

Anastasopoulou

Lekprasert

2021

AACE Clinical Case Reports

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Objective To describe a rare case of pheochromocytoma presenting with hypoglycemia. Methods We describe a rare case of pheochromocytoma presenting with a hypoglycemic seizure. Our article includes our differentials, work up, and management. Results Our patient had non–islet-cell tumor hypoglycemia that was non-insulin mediated, as noted by low insulin levels. His hypoglycemia was likely multifactorial and mediated by different mechanisms. We describe the rare case and review the causes of tumor-induced hypoglycemia. Conclusion Pheochromocytomas can rarely present with hypoglycemia and are associated with a poor prognosis.

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Section: Discussionmentioning

confidence: 99%

Tumor-Induced Hypoglycemia: An Unusual Case Report and Review of Literature

Abdulhadi

Anastasopoulou

Lekprasert

2021

AACE Clinical Case Reports

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“…Direct glucose consumption by the tumor in the presence of an elevated burden of disease, according to the results of the 18F-FDG-PET/CT scan findings. This was reported in a few case reports, with the presence of FDG uptake predominantly in the tumor mass, and the reduction in the normal FDG uptake throughout the rest of the body, most prominently in the brain [ 37 , 38 ];…”

Section: Pathophysiological Mechanisms Of Hypoglycemia Related To Met...mentioning

confidence: 93%

“…The principal mechanisms proposed are subsequently: Direct glucose consumption by the tumor in the presence of an elevated burden of disease, according to the results of the 18F-FDG-PET/CT scan findings. This was reported in a few case reports, with the presence of FDG uptake predominantly in the tumor mass, and the reduction in the normal FDG uptake throughout the rest of the body, most prominently in the brain [ 37 , 38 ]; Excessive ectopic cancer production of aberrant IGF-II or pro-IGF-II, that are less protein-bound and subsequently more bioactive than normal IGF-II. Multiple actions of these compounds can be responsible for the development of hypoglycemia, primarily in the reduction of glucose hepatic output, by decreasing hepatic gluconeogenesis and glycogenolysis.…”

Section: Pathophysiological Mechanisms Of Hypoglycemia Related To Met...mentioning

confidence: 99%

“…Multiple actions of these compounds can be responsible for the development of hypoglycemia, primarily in the reduction of glucose hepatic output, by decreasing hepatic gluconeogenesis and glycogenolysis. On the other hand, abnormal activation of IGF-II of IGF-1 receptors on the pancreatic alpha cells leads to suppression of the production of the insular counter-regulation hormones like GH, IGF-1 and glucagon [ 38 ]; Direct ectopic release of insulin from PGL. Paraganglioma originates from neural cresta cells, and the cosecretion of insulin and catecholamines has been demonstrated by a single case report.…”

Section: Pathophysiological Mechanisms Of Hypoglycemia Related To Met...mentioning

confidence: 99%

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Pathophysiology and Management of Glycemic Alterations before and after Surgery for Pheochromocytoma and Paraganglioma

Lopez

Bima

Bollati

et al. 2023

IJMS

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Glycemic alterations are frequent in patients with pheochromocytoma and paraganglioma (PPGL), but the real incidence of secondary diabetes mellitus (DM) is uncertain, because prospective multicenter studies on this topic are lacking in the literature. The main pathophysiological mechanisms of glucose homeostasis alterations in PPGL, related to catecholamine hypersecretion, are impaired insulin and glucagon-like peptide type 1 (GLP-1) secretion and increased insulin resistance. Moreover, it has been reported that different pathways leading to glucose intolerance may be related to the secretory phenotype of the chromaffin tumor. Predictive factors for the development of glucose intolerance in PPGL patients are a higher age at diagnosis, the need for a higher number of anti-hypertensive drugs, and the presence of secreting neoplasms. Tumor resection is strongly related to the resolution of DM in PPGL patients, with a significant improvement of glycemic control in most cases. We can hypothesize a different personalized therapeutic approach based on the secretory phenotype. The adrenergic phenotype is more closely related to reduced insulin secretion, so insulin therapy may be required. On the other hand, the noradrenergic phenotype mainly acts by increasing insulin resistance and, therefore, insulin-sensitizing antidiabetic agents can find a greater application. Regarding GLP-1 receptor agonists, the data suggest a possible promising therapeutic effect, based on the assumption that GLP-1 secretion is impaired in patients with PPGL. The principal predictors of remission of glycemic alterations after surgery for PPGL are a lower preoperative body mass index (BMI), a larger tumor, higher preoperative catecholamine levels, and a shorter duration of the disease (under three years). Otherwise, after resection of PPGL, hypoglycemia can occur as the result of an excessive rebound of preoperative hyperinsulinemia. It is a rare, but potentially severe complication reported in a lot of case reports and a few small retrospective studies. Higher 24-h urinary metanephrine levels, longer operative times and larger tumors are predictive factors for hypoglycemia in this setting. In conclusion, alterations of carbohydrate metabolism are clinically relevant manifestations of PPGL before and after surgery, but there is the need to conduct multicenter prospective studies to obtain an adequate sample size, and to allow the creation of shared strategies for the clinical management of these potentially severe manifestations of PPGL.

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