Stauffer Syndrome: The Renal-Hepato Syndrome

Abstract

Stauffer's Syndrome is a poorly understood and rare paraneoplastic syndrome in which a reversible cholestasis causes elevation of liver enzymes and hyperbilirubinemia. Hepatomegaly may also be observed. It has most frequently been described in patients with renal cell carcinoma, though can rarely be seen in other malignancies as well. While the association has been noted, the pathogenesis remains elusive as the cholestasis is not related to the presence of metastases or compression/invasion of the biliary tract by closely located tumors. It is theorized that the production of cytokines by the tumor may be responsible for the abnormalities seen. We present a case of a patient whose workup for hyperbilirubinemia led to her diagnosis of renal cell carcinoma. Our patient is a 67 year-old female with a past medical history of diabetes mellitus, hypertension, hyperlipidemia, peptic ulcer disease, iron deficiency anemia, ischemic colitis status post-colostomy, who presented to the ED complaining of progressive shortness of breath and fatigue. She was found to have a hemoglobin of 6.6 on admission, with an iron panel showing a mixed picture of iron deficiency and anemia of chronic disease. On admission, the patient was also found to have an total bilirubin of 1.9 (0.4 direct, 1.5 indirect), alkaline phosphatase to 262, and elevated AST to 153. Hemolysis labs were negative. An abdominal sonogram was performed to evaluate for the presence of cholelithiasis given the abnormal liver function testing. Biliary sludging was seen, no stones were identified. An incidental finding of this scan, however, was a large right-sided renal mass, measuring 22.2 x 12.5 x 20.0 cm. An abdominal CT scan was subsequently performed and identified a 28 centimeter right renal mass. There was no evidence of ductal dilation or obstruction noted. Urology and Nephrology were both consulted, and a biopsy was performed with pathology confirming tumor cells positive for PAX8, CD10, Vimentin, and EMA, which confirmed the patient's diagnosis of clear cell renal cell carcinoma. Stauffer Syndrome is a rare entity that has dire implications. In our patient, the nonspecific LFT elevation and jaundice prompted an initial abdominal ultrasonogram, which led to the diagnosis of renal cell carcinoma. Stauffer syndrome has mainly been described in case reports. This case is unique in that the tumor was initially diagnosed on an abdominal ultrasound.Figure: Right Upper Quadrant U/S showing evidence of Renal Mass on Right Kidney Trans UP Plane.Figure: CT Abdomen/Pelvis confirming presence of large right sided Renal Carcinoma.