Doege — (Potter) the Bullet with a Knife

1Icahn School of Medicine at Mount Sinai, New York, NY

Meeting: Hospital Medicine 2014, March 24-27, Las Vegas, Nev.

Abstract number: 654

Case Presentation:

An 88 year‐old Hispanic man with non‐insulin dependent diabetes, whose oral anti‐hyperglycemic agents were discontinued a week prior to admission due to hypoglycemia, re‐presented with hypoglycemia. He also reported progressive fatigue and poor oral intake for the past few weeks. He lived at home with a home health assistant who helped him with medication management. He was a frail elderly man in no distress. The abdomen was soft and without tenderness or masses. His skin was not icterus and he did not have rashes. Laboratory studies demonstrated normal hepatic function, hepatic transaminases, undetectable sulfonylurea, normal beta‐hydroxybutyrate levels, and a suppressed endogenous insulin level. His infectious work‐up was also negative. Computed Tomography of the abdomen and pelvis revealed a 13.4 x 13.4 x 17.5cm mass extending from the gallbladder neck to the abdomen, without evidence of metastasis. Microscopic examination of the tissue biopsy revealed hypercellularity with up to 6 mitotic figures/10 high power fields. Immunohistochemical studies were positive for CD34, BCL‐2 and CD99. Stains for smooth muscle actin, desmin, S‐100 protein, CD31, FLI‐1, WT‐1 and pancytokeratin are negative. These results are consistent with solitary fibrous tumor (SFT). Serum insulin‐growth factor II (IGF) level was 523ng/ml (high normal) and IGF I was not obtained. While hospitalized, the patient remained persistently hypoglycemic, requiring continuous dextrose‐10% infusion. He underwent cholecystectomy with complete removal of the mass. Shortly after surgery, he was weaned off the dextrose infusion.

Discussion:

Hypoglycemia is one of the most common problems encountered by hospitalists and malignancy should always be included in the differential diagnosis of persistent hypoglycemia. This patient’s presentation and findings are most consistent with Doege‐Potter Syndrome (DPS). DPS is a rare paraneoplastic syndrome characterized by a solitary fibrous tumor SFT secreting IGF‐II, causing hypoglycemia. The diagnosis in this case was based on findings of a large‐size SFT – SFTs larger than 8‐10cm have increased risk for becoming paraneoplastic ‐, low insulin level while hypoglycemic and the exclusion of other causes of low‐insulin hypoglycemia. Available commercial assays for IGF II level often yield a normal level, while a ratio of IGF II/IGF I >10 is more diagnostic. SFTs typically originate from intrathoracic pleura, but rarely could be extrapleural. In a large retrospective study of 4000 soft tissue tumors obtained over 18 years, only 79 SFTs were identified, 54 of those were intrathoracic in origin. Paraneoplastic syndrome causing hypoglycemia, DPS, is less than 5% of SFT cases. Surgical resection is currently the definitive treatment.

Conclusions:

Paraneoplastic syndrome should be considered in a patient with persistent hypoglycemia and surgical resection could be curative as in this case.

To cite this abstract:

Truong T, Lin L. Doege — (Potter) the Bullet with a Knife. Abstract published at Hospital Medicine 2014, March 24-27, Las Vegas, Nev. Abstract 654. Journal of Hospital Medicine. 2014; 9 (suppl 2). https://www.shmabstracts.com/abstract/doege-potter-the-bullet-with-a-knife/. Accessed July 22, 2019.

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