Case Report
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Sep 6, 2022; 10(25): 9087-9095
Published online Sep 6, 2022. doi: 10.12998/wjcc.v10.i25.9087
Renal cell carcinoma presented with a rare case of icteric Stauffer syndrome: A case report
Dimitar R Popov, Krasimir A Antonov, Evelina G Atanasova, Christo P Pentchev, Lyuben M Milatchkov, Maria D Petkova, Krasimir G Neykov, Rosen K Nikolov
Dimitar R Popov, Krasimir A Antonov, Christo P Pentchev, Maria D Petkova, Rosen K Nikolov, Medical University of Sofia, Sofia 1431, Bulgaria
Dimitar R Popov, Krasimir A Antonov, Evelina G Atanasova, Christo P Pentchev, Lyuben M Milatchkov, Maria D Petkova, Rosen K Nikolov, Clinic of Gastroenterology, University Hospital ’St. Ivan Rilski’, Sofia 1431, Bulgaria
Krasimir G Neykov, Clinic of Urology, National Oncology Hospital, Sofia 1756, Bulgaria
Author contributions: Popov DR, Antonov KA, and Nikolov RK conceived and supervised the study; Atanasova EG, Pentchev CP, Milatchkov LM, and Petkova MD collected the clinical data and followed up with the patient; Popov DR and Antonov KA analyzed the data; Neykov KG contributed to the nephrectomy procedures; Pentchev CP, Atanasova EG, Milatchkov LM, and Petkova MD wrote the paper; all authors have read and approved the final manuscript.
Informed consent statement: The authors declare that the person involved in the case report gave his informed consent to be involved in the study. The informed consent has been provided to the publisher.
Conflict-of-interest statement: The authors declare that there are no conflicts of interest associated with this work.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Evelina G Atanasova, MD, PhD, Staff Physician, Clinic of Gastroenterology, University Hospital ‘St. Ivan Rilski’, ‘Akademik Ivan Evstratiev Geshov’ Blvd. No. 15, Sofia 1431, Bulgaria. eva_gatanasova@abv.bg
Received: April 2, 2022
Peer-review started: April 2, 2022
First decision: May 31, 2022
Revised: June 16, 2022
Accepted: August 1, 2022
Article in press: August 1, 2022
Published online: September 6, 2022
Processing time: 145 Days and 14.6 Hours
Abstract
BACKGROUND

Paraneoplastic syndromes remain poorly understood and manifest as multifaceted clinical symptoms, making their diagnosis difficult. Cholestasis can be observed in various malignancies. In rare cases, it can be a paraneoplastic manifestation, most often associated with renal cell carcinoma and other urogenital tumors, as well as with bronchial carcinoma. The classical form of Stauffer syndrome presents with a reversible anicteric increase of cholestatic liver function tests, thrombocytosis, coagulation impairment, and hepatosplenomegaly, without any proven hepatobiliary obstruction or metastases.

CASE SUMMARY

We report a patient who presented with elevated liver enzymes, cholestatic jaundice, weight loss and pruritus, in whom renal cell carcinoma was incidentally found during hospitalization. Clinical, laboratory, and imaging tests excluded primary hepatic cause or metastatic disease. Jaundice and laboratory abnormalities reversed completely a few months after nephrectomy. This case is an example of the many sides of renal cell carcinoma, and it focuses the clinicians’ attention on the differential diagnosis of cholestasis, including Stauffer syndrome and its variant. Thus, the correct diagnosis can be straightforward and the associated malignancy can be treated promptly. All cases should be followed up with a multidisciplinary team. Interleukin (IL)-6 is proposed to contribute to the pathophysiology of the condition. The probable mechanism is proinflammatory activity by the IL-6 cytokine, causing elevation of C-reactive protein and haptoglobin and inhibition of hepatobiliary transporter gene expression, impairing biliary outflow.

CONCLUSION

Despite being rare, Stauffer syndrome is a potentially reversible paraneoplastic condition, when the primary cause is treatable. This syndrome should be considered by clinicians because of the remediable liver disturbance, after successful treatment of the underlying malignancy.

Keywords: Jaundice; Cholestasis; Renal cell carcinoma; Stauffer syndrome; Case report

Core Tip: Initial presentation of jaundice, in cases of non-hepatic malignancy, is generally associated with metastatic liver disease. Despite the increased bilirubin levels, surgical treatment, when possible, is an appropriate approach in a case with non-metastatic cholestatic syndrome. Stauffer syndrome is a rare presentation of kidney and urinary tract malignancies and even rarer when presented with jaundice.