Stauffer Syndrome: A Comprehensive Review of the Icteric Variant of the Syndrome

Stauffer syndrome is a rare paraneoplastic syndrome classically associated with renal cell carcinoma (RCC). This association gave it the historical name of nonmetastatic nephrogenic hepatic dysfunction syndrome without jaundice. It is a syndrome of unclear pathophysiology characterized by a reversible anicteric elevation of liver enzymes, alkaline phosphatase, erythrocyte sedimentation rate (ESR), thrombocytosis, prolongation of prothrombin time, and hepatosplenomegaly in the absence of direct hepatobiliary obstruction or jaundice. A rare atypical variant of this syndrome with jaundice has been recently described in the literature. Thus, it is important to consider both these variants of Stauffer syndrome in the differential diagnosis of unexplained cholestasis in the absence of hepatic metastasis. This may allow early recognition and treatment of an occult malignancy. Herein, we present a comprehensive review of the literature available on the icteric variant of the Stauffer syndrome, outlining its association with various malignancies and the diagnostic challenges it poses. The objective of this review is to help clinicians in its early diagnosis and management.