Disorders of the Pleura: Student/Resident Case Report Poster - Disorders of the Pleura |

A Rare Paraneoplastic Syndrome Causing Hypoglycemia FREE TO VIEW

Sunya Ashraf, MD; Niktha Kasinathan, MD; Stephen Abo, DO
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Hackensack UMC Mountainside Hospital, Lyndhurst, NJ

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):591A. doi:10.1016/j.chest.2016.08.680
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SESSION TITLE: Student/Resident Case Report Poster - Disorders of the Pleura

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Hypoglycemia can often times be the manifestation of a tumor related paraneoplastic syndrome. Most commonly, the underlying malignancy is an insulinoma or hepatocellular carcinoma. It can also be associated with fibrosarcomas or other rare mesenchymal tumors, such as a solitary fibrous tumor of the pleura (SFTP). SFTP is a rare, slow growing tumor that arises from mesenchymal tissue of the pleura that accounts for approximately 5% of pleural tumors, with a 12-13% malignancy rate. This case will describe the presentation and management of this disease and a rare hypoglycemic syndrome associated with it.

CASE PRESENTATION: 66 year old non-diabetic male, history of malignant SFTP presented to the emergency room with symptomatic hypoglycemia and a blood sugar of 24. His symptoms resolved with dextrose ampules, however he remained severely hypoglycemic while receiving continuous dextrose infusions. He was diagnosed with SFTP in 2004 for which he underwent three pleurectomies and adjuvant external beam radiation due to recurrence. After progressive disease, he was treated with bevacizumab/temozolamide, followed by pazaponib. His most recent CT scan revealed three large pleural based masses measuring up to 10 cm with retroperitoneal extention. Physical exam was remarkable for decreased breath sounds throughout left lung field. Lab testing revealed a low C-peptide and insulin concentration excluding insulinoma, ectopic insulin production or exogenous insulin administration. Serum cortisol, adrenocorticotrophic hormone, and growth hormone levels were also normal, and no insulin antibody was detected (Table 1). Therefore the diagnosis of Doege Potter Syndrome was made. Due to the extent of his disease, he was not a surgical candidate. His sugars remained stable with frequent meals, and snacks throughout the night. Decadron was initiated and his pazaponib was continued. His Family was educated on signs and symptoms of hypoglycemia and emergency glucagon usage.

DISCUSSION: Doege-Potter Syndrome is a rare paraneoplastic syndrome that occurs in SFTP. The hypoglycemia is due to overproduction of incompletely processed insulin-like growth factor (IGF)-II, resulting in the stimulation of the insulin receptors and increased glucose utilization. Hypoglycemia occurs in only 4% of cases, usually associated with large volume tumors which typically resolves with surgery.

CONCLUSIONS: Unfortunately, our patient has non-resectable disease and systemic therapies for SFTP have poor response rates. Our case highlights how we foresee hypoglycemic episodes persisting in our patient.

Reference #1: Fukuda I, et al. Clinical features of insulin-like growth factor-II producing non-islet-cell tumor hypoglycemia. Growth Horm IGF Res. 2006;16:211-216.

Reference #2: Schutt, Robert C., et al, “Doege-Potter Syndrome Presenting with Hypoinsulinemic Hypoglycemia in a Patient with a Malignant Extrapleural Solitary Fibrous Tumor: A Case Report.” J Med Case Rep Journal of Medical Case Reports 7.1 (2013): 11. Web.

DISCLOSURE: The following authors have nothing to disclose: Sunya Ashraf, Niktha Kasinathan, Stephen Abo

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