P2.04-037 Solitary Fibrous Tumor of the Pleura Associated with Severe Hypoglicemia: The Doege-Potter Syndrome: Topic: Esophageal Cancer and Other Malignancies

Abstract

to present an extremely rare case of Doege-Potter Syndrome (DPS). a 66 years-old man, non-smoker, was admitted for disorientation and confusional state. Blood tests showed a severe hypoglycemia (30 mg/dl); the patient was not diabetic and did not take antihyperglycemic agents. An extremely low values of insulin and C-peptide was also found. A chest CT-scan showed a 20x16cm lobulated mass occupying the right hemithorax (Figure 1A). FDG-PET showed a unique uptake of the lesion (SUVmax 2.5). After a multidisciplinary discussion, a surgical resection was proposed. The lesion of pleural origin was radically resected through a right posterolateral thoracotomy. From the 1st postoperative day, the blood glucose levels were normal; the patient was discharged on the 5th postoperative day uneventfully. the mass measured 26×18×12cm, presented polylobate margins, elastic consistency and grayish-white color (Figure 1B). Microscopically, a mesenchymal neoplasm characterized by spindle cells with moderate atypia, hyperchromasia and nuclear pleomorphism, organized in a fascicular pattern was found (Figure 1C). A moderate deposition of collagen extracellular matrix, with areas of hyaline involution and foci of necrosis was evident (Figure 1D). The mitotic index was 9x10 HPF. Immunohistochemically, tumor cells were positive for CD34, CD99, BCL-2, vimentin, desmin, IGF1-R, IGF-2, Ki67 (15%) and negative for EMA, smooth muscle actin, cytokeratin pool, c-kit, DOG-1, myogenin, calretinin, S100. Considering these histopathologic features, a diagnosis of malignant solitary fibrous tumor of the pleura was made. After 18 months, the patient is in good condition and free of disease. DPS is a paraneoplastic syndrome characterized by severe hypoglycemia associated with a solitary fibrous tumor of the pleura. Surgical therapy is the gold standard, providing also the resolution of hypoglycemic crisis. Close follow-up and blood glucose monitoring is essential, especially in patients suffering from the malignant variant of this rare pleural tumor.