Endocrine Abstracts

Introduction: 2 to 3% of the causes of hypoglycemia in the population without diabetes mellitus correspond to tumors of cells other than islets. Below is a rare case of hypoglycemia related to a pleural tumor.

Clinical case: A 65-year-old woman with arterial hypertension, psychiatric treatment for confusion and behavioral changes of 6 months of evolution, palpitations and night sweats, loss of 20 kg of weight, several admissions to the emergency room, until her hospitalization due to alteration of the sensory, where severe hypoglycemia is found. She denies using hypoglycemic agents, corticosteroids, and alcoholism. Physical examination: Diminished vesicular murmur in the middle and lower left lung fields. Laboratory tests: Plasma glucose: 30 mg/dl, serum insulin: less than 1 Uu/ml, C peptide: 0.17 ng/ml, anti-insulin antibodies: 1.8 negative, the rest in normal range including cortisol, normal thyroid, renal and hepatic function. Chest CT: left pleural tumor of 30 x 20 cm in the left hemithorax. Biopsy: spindle cell proliferation. Tumor excision and histology: well-defined stromal neoplastic proliferation, without atypia, suggestive of an intense, diffuse, cytoplasmic CD34+ solitary fibrous tumor. Desmin, pancytokeratin AE1/AE3 and S 100 negative, ki67 10%, resolved hypoglycemia after surgery.

Discussion: Doege- Potter syndrome is a rare cause of hypoglycemia, characterized by solitary fibrous tumors that are associated with hypoglycemia of non-islet cell tumors of the pancreas (NICTH). These are epithelial of mesenchymal tumors, of various locations, that produce IGF2, which bind to insulin receptors, increasing peripheral glucose consumption, decreasing glycogenolysis and gluconeogenesis. In general, hypoglycemia is fasting with confusional symptoms; as the case of our patient; in which it is confused with a psychiatric picture. Decreased peptide C and decreased insulinemia make it necessary to think about NICTH. In half of the cases, hypoglycemia precedes the discovery of tumors, as stated. Hypoglycemia is not related to aggressiveness or tumor size. Random IGF2 levels may be normal; in this case IGF2 dosing was not possible. Complete resolution of hypoglycemia after surgery supports the diagnosis of the syndrome. In conclusion, Doege- Potter syndrome, a rare and potentially reversible entity, should be evaluated in the differential diagnosis of hypoglycemia in the population without diabetes.