Introduction: Stauffer syndrome (SS) is a paraneoplastic syndrome seen in some malignancies, most commonly 4-15% of primary renal cell carcinomas, resulting in nephrogenic hepatic dysfunction. SS commonly results in elevated alkaline phosphatase, alanine aminotransferase, aspartate aminotransferase, and prothrombin time (PT) as well as decreased albumin in the absence of liver metastasis. The etiology of SS is unknown, although three have been suggested: elevated interleukin-6, elevated colony stimulating factor or, least likely, autoimmune. We describe a case of a woman presenting with right upper quadrant (RUQ) pain who was found to have indirect predominant hyperbilirubinemia and acute cholecystitis and renal mass on ultrasound.
Case Description/Methods: An 81-year-old female presented to an outside hospital for RUQ and epigastric pain that worsened after fatty meals. Imaging showed acute cholecystitis as well as a right renal mass. Physical exam at the time was remarkable only for diffuse tenderness to abdominal palpation, most notably in the RUQ. There was no scleral icterus or costovertebral angle tenderness on exam. Labs on presentation can be seen in Table 1.
Given the elevated indirect bilirubin, gastroenterology was consulted and suggested Gilbert syndrome (GS) or SS given their concern for renal cell carcinoma (RCC). The next day the patient underwent a laparoscopic cholecystectomy notable for acute gangrenous cholecystitis with cholelithiasis, and had rapid normalization of bilirubin over 48 hours. The patient followed with outpatient urology for renal biopsy, which was positive for clear cell RCC. Follow up imaging revealed scattered pulmonary nodules up to 1.1cm, with concern for metastasis. Surgery was deemed inappropriate and the patient was conservatively managed, given her wishes to prioritize quality of life and not undergo chemotherapy.
Discussion: This case illustrates an interesting clinical picture where a patient with confirmed cholecystitis also has a new renal mass, with elevated indirect bilirubin and PT, consistent with SS. The diagnosis of SS is made clinically, with hopeful resolution of abnormal lab values upon RCC resection. In this case, surgery was not indicated, hemolysis was ruled out with stable hemoglobin and normal peripheral smear, and the diagnoses of acute cholecystitis and RCC causing SS was made. In addition to hemolysis, GS, and Criggler-Najjar when working up indirect hyperbilirubinemia, it is imperative to include SS on the differential diagnosis.
