Doege-Potter syndrome presenting with hypoinsulinemic hypoglycemia in a patient with a malignant extrapleural solitary fibrous tumor: a case report

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.

Bladder urothelial carcinoma producing insulin‐like growth factor II: A case report

“…To the best of our knowledge, this is the first description of a case of IGF-IIproducing bladder urothelial carcinoma associated with NICTH, although a few cases of NICTH caused by solitary fibrous tumor of the bladder have been reported. 7 The PDX model established in the present study preserved histological characteristics of original tumor as well as strong IGF-II expression, which helped better understanding of this rare pathogenic entity. PDX models recently attract considerable attention 3 particularly for the researches on certain cancers that have been hampered by the shortage of available animal models.…”

“…Instead, we detected it in PDX tumor and thereby confirmed the diagnosis of NICTH in the present case. To the best of our knowledge, this is the first description of a case of IGF‐II‐producing bladder urothelial carcinoma associated with NICTH, although a few cases of NICTH caused by solitary fibrous tumor of the bladder have been reported …”

Case Report: Doege-Potter Syndrome.

“…Other treatment option involves chemotherapy. Treatment for hypoglycemic attacks include scheduled snacks, nocturnal or continous dextrose infusion or enteral tube feeding, corticosteroids, continous glucagon infusion [12]. Doege Potter syndrome should be considered in patients who have intra-thoracic tumour and recurrent hypoinsulinemic hypoglycemia.…”

Doege–Potter Syndrome

“…The resultant hypoglycaemia can be fatal if definitive surgical intervention is not performed. 2,6 SFTs are mesenchymal neoplasms of unknown derivation; they are commonly of thoracic origin but extrathoracic cases are also seen. 6 Despite this anatomical heterogeneity, SFTs behave as a single biological entity with shared clinical and pathological features.…”