ECEESPE2025 ePoster Presentations Endocrine Related Cancer (100 abstracts)
1Hospital Universitario de Toledo, Laboratory Medicine, Toledo, Spain; 2Hospital Clínico Universitario Lozano-Blesa, Neurosurgery, Toledo, Spain; 3Hospital Universitario de Toledo, Endocrinology, Toledo, Spain
JOINT1161
Introduction: Doege-Potter syndrome is a rare clinical entity characterized by recurrent hypoglycemic events caused by non-pancreatic tumors secreting an incompletely processed high-molecular-weight form of Insulin-like Growth factor-II (IGF-II). Usually caused by a solitary fibrous tumor (SFT) and mediated by the binding of IGF-II precursors to insulin receptors. They have a benign behavior in most cases and rarely produce metastasis, although up to 12% present malignant behavior. They are more frequent in men, between the sixth and eighth decade of life and predominate in the right hemithorax. The treatment of choice is surgery, but in unresectable cases, although glucocorticoids (GC) play a major role, the approach is controversial. Our aim is to present a case of Doege-Potter syndrome and the importance of the IGF-II/IGF-I ratio in individuals with hypoglycemia secondary to IGF-II-producing STF.
Case report: A 65-year-old male diagnosed with pleural SFT. He was admitted to the Emergency Department due to massive pleural effusion secondary to a large mass in the left hemithorax (> 20 cm) and extrapulmonary extension. Clinical signs of IGF-II-mediated hypoglycemia (<20 mg/dL): C-peptide 0.19 ng/mL, insulin <0.2 uU/mL, IGF-I 53.3 ng/mL (93-224), IGF-II 941 ng/mL (350-1000), IGF-II/IGF-I 17.6 (VN <10). Treatment with GC was started, requiring escalation to 1.5 mg/kg/day and glucagon to prevent hypoglycemia, so diazoxide was added to reduce the GC dose. Continuous interstitial glucose monitoring (CGM) and selective radiotherapy (SR) were started which facilitated management and subsequent surgical resection.
Discussion and conclusions: Hypoglycemia secondary to large IGF-II-producing STF is a therapeutic challenge. CGM is an aid in management, as well as a response to diazoxide and SR described in the literature to achieve tumor control and hypoglycemia that allows curative surgical treatment. However, a recurrence rate of up to 15% is estimated according to described series. These are slow-growing tumors with few initial respiratory symptoms and in which hypoglycemia may be the initial symptom, so emphasis should be placed on the evaluation of thoracic imaging studies in the event of persistent hypoglycemia. The plasma IGF-II/IGF-I ratio better indicates the Doege-Potter syndromes metabolic impairment than isolated measurements of circulating IGF-II or IGF-I levels.