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

BackgroundAdrenocortical carcinoma (ACC) is a rare malignancy arising from the adrenal cortex. While ACC can be associated with adrenal hormone excess syndromes, classic paraneoplastic syndromes are rarely seen. Stauffer syndrome, a paraneoplastic phenomenon characterized by reversible cholestasis i...

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Bibliographic Details
Published in:Journal of the Endocrine Society 2022, Vol.6 (8), p.bvac101-bvac101
Main Authors: Murvelashvili, Natia, Polanco, Patricio M, Khorsand, Sarah M, Marrero, Jorge A, Jia, Liwei, Mirfakhraee, Sasan, Else, Tobias, Habra, Mouhammed Amir, Cole, Suzanne, Hamidi, Oksana
Format: Report
Language:English
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Summary:BackgroundAdrenocortical carcinoma (ACC) is a rare malignancy arising from the adrenal cortex. While ACC can be associated with adrenal hormone excess syndromes, classic paraneoplastic syndromes are rarely seen. Stauffer syndrome, a paraneoplastic phenomenon characterized by reversible cholestasis in the absence of liver metastases, has been described with renal carcinoma and other malignancies but has not been previously reported in ACC. Case PresentationA 38-year-old man presented with emesis, painless jaundice, pruritus, and weight loss. Laboratory evaluation demonstrated elevated total bilirubin of 8.7 mg/dL (N 
ISSN:2472-1972
DOI:10.1210/jendso/bvac101