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A6702 - Doege Potter Syndrome: Solitary Fibrous Tumor of the Pleura Associated with Hypoglycemia
Author Block: M. I. Maguina1, D. A. Salazar Martinez2, G. F. Velasquez Pinto3, W. Chavez3, A. Ventura1, O. Flores1, C. Colunche3, O. Gayoso4, J. F. Ramirez5, J. F. Ramirez3; 1Lima, Hospital Cayetano Heredia, San Martín de Porres, Peru, 2Pulmonology, Hospital Cayetano Heredia, Lima, Peru, 3Hospital Cayetano Heredia, Lima, Peru, 4HOSPITAL CAYETANO HEREDIA, Lima, Peru, 5Cleveland Clinic Florida, Fort Lauderdale, FL, United States.
Doege-Potter Syndrome: Solitary Fibrous Tumor of the Pleura Associated with Hypoglycemia.
Authors
M. Maguina-Farronay, D.A. Salazar, G. Velasquez, W. Chavez, A. Ventura, O. Flores, C. Colunche, O. Gayoso, J. Ramirez- Ramirez.
Introduction
The Doege-Potter syndrome is a rare paraneoplastic syndrome. It consists of the association of hypoglycemia with a solitary fibrous tumor of the pleura. The solitary fibrous tumor is a slow growing neoplasm that arises from the mesenchymal cells of the submesothelial layer of the pleura. Hypoglycemia is secondary to tumor secretion of an aberrant form of Insulin-like growth factor 2, a peptide that share structural similarity to insulin and permanently stimulates the insulin receptors.
Case Presentation
A 60-year-old Andean woman, came to the emergency room with a 4-month history of loss of appetite, weight loss and progressive drowsiness. On admission, the patient was unresponsive. On physical examination the patient was pale, sweaty and tachycardic; vesicular breath sounds were abolished at the left lung base, GCS: 9.
Capillary glucose was 21mg/dL. She was treated immediately with boluses of 33% dextrose and 10% dextrose IV infusion. The patient recovered conscience, but she had several recurrent episodes of hypoglycemia despite of the dextrose infusion.
Chest X- ray showed a large mass on the left lung base displacing the mediastinum. Chest CT scan showed a homogeneous well-defined large mass at the left lower lobe.Lab studies showed: C peptide: 0.15 ng/dl, Insulin: 1.6 uU/ml, cortisol: 11.7mg/dl, IGF-1: 51 ng/ml.
A percutaneous lung biopsy was performed. The pathology showed a thickened pleura occupied by fusiform cells arranged in a storiform pattern, with atypical and hyperchromatic cells between a dense collagen. These findings were suggestive of solitary fibrous tumor.
A surgical resection of the tumor was performed. A large mass firmly attached to the diaphragm was found. The mass was 21x20x9 cm and weighed 1400 mg. The pathology was consistent of solitary fibrous tumor and the immunohistochemistry showed: BCL2: Positive, CD34: Positive, Ki67: 2% approximately.
After the surgery the patient had stable values of glycaemia and the dextrose infusion was stopped. This confirmed the diagnosis of Doege-Potter Syndrome. However, the patient died on the third day post-operative from surgical complications.
Discussion
The patient presented with severe episodes of hypoglycemia associated to a large asymptomatic intrathoracic mass. The final diagnosis was done by confirming the pathology and the immunochemistry of the solitary fibrous tumor and the correction of the hypoglycemia after the removal of the mass.