From the Guidelines
Stauffer syndrome is a paraneoplastic condition characterized by signs of cholestasis unrelated to tumor infiltration of the liver or intrinsic liver disease, which typically resolve after kidney tumor resection, as noted in the 2019 ESMO clinical practice guidelines for renal cell carcinoma diagnosis, treatment, and follow-up 1. This condition is primarily associated with renal cell carcinoma and can present with elevated liver enzymes, jaundice, hepatomegaly, and abnormal liver function tests despite having no direct tumor involvement of the liver.
- The exact mechanism is believed to involve inflammatory cytokines released by the tumor that affect liver function.
- The syndrome may also include fever, weight loss, and fatigue.
- Importantly, Stauffer syndrome often resolves after the primary kidney tumor is removed through nephrectomy, making it a potentially reversible condition, as stated in the guidelines 1.
- No specific medication regimen exists for Stauffer syndrome itself; treatment focuses on addressing the underlying renal cell carcinoma.
- Early recognition of this syndrome is valuable as it can sometimes be the first indication of an otherwise asymptomatic kidney cancer, allowing for earlier diagnosis and treatment of the primary malignancy. Key laboratory examinations for suspected renal cell carcinoma, which may also aid in the diagnosis of Stauffer syndrome, include serum creatinine, haemoglobin, leukocyte and platelet counts, lymphocyte to neutrophil ratio, lactate dehydrogenase, C-reactive protein (CRP), and serum-corrected calcium, as recommended in the guidelines 1.
From the Research
Definition and Characteristics of Stauffer Syndrome
- Stauffer syndrome is a rare paraneoplastic syndrome classically associated with renal cell carcinoma (RCC) 2, 3, 4, 5, 6.
- It is 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 2.
- A rare atypical variant of this syndrome with jaundice has been described in the literature 2, 3, 5.
Association with Malignancies
- Stauffer syndrome is associated with various malignancies, particularly renal cell carcinoma 2, 3, 4, 5, 6.
- It has also been described in lymphoproliferative diseases, prostate cancer, and broncogenic tumors 5.
Diagnostic Challenges and Treatment
- The syndrome poses diagnostic challenges, and it is essential to consider both the icteric and anicteric variants in the differential diagnosis of unexplained cholestasis in the absence of hepatic metastasis 2.
- Proper treatment of the underlying disease can lead to the resolution of paraneoplastic signs and symptoms of Stauffer's syndrome 5, 6.
- Treatment regimens, including surgery and immunotherapy, have been employed in cases of Stauffer's syndrome, with some cases showing improvement or resolution of symptoms 4, 6.