Stauffer syndrome: a comprehensive review of the disease and diagnostic plan proposal

Abstract

Stauffer syndrome, first described by Herbert Stauffer in 1961, is a hepatic paraneoplastic syndrome characterized by multiple extrahepatic malignancies, most commonly renal cell carcinoma. The syndrome manifests a wide range of symptoms caused by various pathophysiological mechanisms and presents with abnormalities in liver function tests in either cholestatic or non-cholestatic patterns.Stauffer’s syndrome is classified into two types: classical and jaundice variants. Some crossovers continue to occur, complicating the diagnosis of such a rare and frequently missed syndrome, which can be the only diagnostic clue for the retrograde detection of a hidden malignancy.To bridge the gap regarding such an important, but still unrecognized, syndrome, not only did we thoroughly cover what had previously been proposed in the literature, but we also proposed a diagnostic protocol based on multicenter experience with such a rare disease.