Abstract
IntroductionDoege-Potter syndrome is a paraneoplastic syndrome characterized by non-islet cell tumor hypoglycemia secondary to a solitary fibrous tumor. This tumor causes hypoglycemia by the secretion of a prohormone form of insulin-like growth factor II. We describe the diagnosis and management of Doege-Potter syndrome and the use of transarterial chemoembolization in a patient with a malignant extrapleural solitary fibrous tumor.Case presentationOur patient was a 64-year-old Caucasian woman who initially presented with urinary incontinence and was found to have a 14.5×9.0×9.0cm retroperitoneal solitary fibrous tumor compressing her bladder. Her tumor was surgically resected but recurred with multiple hepatic metastatic lesions. The hepatic metastases progressed despite systemic chemotherapy and treatment with doxorubicin transarterial chemoembolization. Her course was complicated by the development of recurrent fasting hypoglycemia, most likely secondary to Doege-Potter syndrome. Her hypoglycemia was managed with corticosteroid therapy and frequent scheduled nutrient intake overnight.ConclusionsThe rarity of hepatic solitary fibrous tumors and consequent lack of controlled trials make this report significant in that it describes the diagnostic approach to Doege-Potter syndrome, describes our experience with the use of doxorubicin transarterial chemoembolization, and presents management options for tumor-associated hypoglycemia in the case of extensive disease not amenable to surgical resection.
Highlights
Doege-Potter syndrome is a paraneoplastic syndrome characterized by non-islet cell tumor hypoglycemia secondary to a solitary fibrous tumor
The rarity of hepatic solitary fibrous tumors and consequent lack of controlled trials make this report significant in that it describes the diagnostic approach to Doege-Potter syndrome, describes our experience with the use of doxorubicin transarterial chemoembolization, and presents management options for tumor-associated hypoglycemia in the case of extensive disease not amenable to surgical resection
Doege-Potter syndrome [1,2] is a paraneoplastic syndrome characterized by non-islet cell tumor hypoglycemia (NICTH) secondary to a solitary fibrous tumor (SFT) that secretes a prohormone form of insulin-like growth factor II (IGF-II)
Summary
Doege-Potter syndrome is an uncommon paraneoplastic phenomenon associated with SFT characterized by hypoinsulinemic hypoglycemia. The diagnosis of Doege-Potter syndrome in our patient was based on the large size of the SFT, low insulin level measured while hypoglycemic, and elevated IGF-II:IGF-I ratio. Her hypoglycemia was managed with daily corticosteroid therapy and frequent nutrient intake, with frequent alarm reminders overnight. HPC and SLC reviewed the pathology, made critical revisions for content, and provided figures for the manuscript. Author details 1Department of Internal Medicine, University of Virginia, PO Box 800696, Charlottesville, VA 22908, USA. Author details 1Department of Internal Medicine, University of Virginia, PO Box 800696, Charlottesville, VA 22908, USA. 2Department of Internal Medicine, Division of Endocrinology and Metabolism, University of Virginia, Charlottesville, USA. 3Department of Pathology, University of Virginia, Charlottesville, USA. 4Department of Internal Medicine, Division of Hematology and Oncology, University of Virginia, Charlottesville, USA
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