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PSAT010 Severe Cholestatic Jaundice (Stauffer Syndrome) as a Rare Paraneoplastic Manifestation in Adrenocortical Carcinoma

Authors :
Natia Murvelashvili
Patricio Polanco
Sarah Khorsand
Jorge Marrero
Liwei Jia
Sasan Mirfakhraee
Tobias Else
Mouhammed Habra
Suzanne Cole
Oksana Hamidi
Source :
Journal of the Endocrine Society. 6:A90-A91
Publication Year :
2022
Publisher :
The Endocrine Society, 2022.

Abstract

Introduction Adrenocortical Carcinoma (ACC) is a rare and often aggressive malignancy arising from the adrenal cortex. Rarely, ACC can be associated with a paraneoplastic syndrome, such as tumor-associated hypoglycemia due to insulin-like growth factor-2 secretion, hyperreninemic hyperaldosteronism, erythropoietin-associated polycythemia, and leukocytosis due to chemokine release from the tumor. Stauffer syndrome is a paraneoplastic syndrome characterized by reversible cholestasis in the absence of liver metastases, most frequently described in the context of renal cell carcinoma. Our case is the first reported association between Stauffer syndrome and ACC. Clinical Case A 38-year-old man presented with nausea, vomiting, painless jaundice, pruritus, and weight loss. Laboratory evaluation revealed an elevated total bilirubin of 8.7 mg/dL (N Conclusion Our case highlights a unique presentation of paraneoplastic hepatic dysfunction with jaundice associated with newly diagnosed ACC. Although Stauffer syndrome is one of the most characteristic paraneoplastic syndromes associated with renal cell carcinoma and other malignancies, paraneoplastic hepatic dysfunction associated with ACC has not been previously reported. The patient had rapid improvement of hyperbilirubinemia after surgical resection of the tumor. Stauffer syndrome should be considered in patients with ACC with liver dysfunction and jaundice without evidence of liver metastases. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.

Details

ISSN :
24721972
Volume :
6
Database :
OpenAIRE
Journal :
Journal of the Endocrine Society
Accession number :
edsair.doi...........a18342d7a27635d0e311d68c3e044221
Full Text :
https://doi.org/10.1210/jendso/bvac150.186