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

Neha Sharma,Gagan Sood,Amir Darr,Umar Darr

doi:10.7759/cureus.6032

Neha Sharma, Gagan Sood + Show 2 more

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https://doi.org/10.7759/cureus.6032

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Abstract

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.

Highlights

BackgroundStauffer syndrome, known as Block‐Stauffer‐Rothmand's Syndrome, Thomson‐Rothmand's Syndrome, ‘nephrogenous hepatosplenomegaly’ or ‘nephrogenous hepatic dysfunction’ was first described by an American gastroenterologist Maurice H
Stauffer syndrome is a rare paraneoplastic syndrome classically associated with renal cell carcinoma (RCC)
He noted that patients suffering from renal cell carcinoma (RCC) had abnormal liver function tests, hepatosplenomegaly, and nonspecific hepatitis type histologic changes in the absence of hepatic metastasis

Summary

Introduction

Known as Block‐Stauffer‐Rothmand's Syndrome, Thomson‐Rothmand's Syndrome, ‘nephrogenous hepatosplenomegaly’ or ‘nephrogenous hepatic dysfunction’ was first described by an American gastroenterologist Maurice H. This led to the conclusion that hepatic dysfunction in these patients was of a paraneoplastic nature This syndrome has been classically described in relation to the early stages of renal cell cancer, but over time, literature has emerged supporting its association with other cancers including bronchogenic carcinoma, bladder cancer, pancreatic cancer, metastatic prostate adenocarcinoma, soft tissue sarcoma, and malignant lymphoproliferative diseases. It can be concluded that IL-6 plays a central role in the pathogenesis of Stauffer syndrome This explains why patients with elevated levels of serum IL‐6 and CRP have elevated levels of haptoglobin, bilirubin, gamma glutamyl‐ transferase, platelets, polymorphonuclear neutrophils (PMN), and alkaline phosphatase. Other laboratory abnormalities include elevation of ESR, alpha2-globulin, gamma-glutamyl transferase, thrombocytosis, and prolongation of prothrombin time [1, 6] With these findings, patients are often worked up for a hepatic source. This syndrome must be differentiated from jaundice caused by metastatic infiltration of the liver, which carries a considerably worse prognosis [5]

Conclusions

Findings

Disclosures