Scleroderma associated with renal cell carcinoma: A case report and literature review

Abstract

Introduction: Systemic sclerosis (aka scleroderma) is an autoimmune disease with no known definitive etiology, but genetic, infectious, and non-infectious exposures have been associated with its occurrence. Previous studies have shown that systemic sclerosis can be a paraneoplastic phenomenon and may be associated with increased risk of malignancy, most commonly lung, skin, and breast cancers. The association of renal cell carcinoma with systemic sclerosis is rare. Case Description: Here, we present a case of a 75-year-old male patient with a rapidly progressive scleroderma despite the initial treatment with methotrexate and prednisone 5 mg daily. Shortly after the diagnosis of scleroderma, the patient was found to have a renal mass. The patient underwent a right partial nephrectomy revealing papillary renal cell carcinoma. The surgical margin was negative indicating complete removal of the cancer. The patient, later, developed scleroderma renal crisis and progressed to end-stage renal disease despite treatment with captopril, mycophenolate mofetil, plasmapheresis, and intravenous immunoglobulin. Conclusions: The purpose of this case report and literature review is to highlight that diffuse scleroderma can potentially be paraneoplastic from a renal cell carcinoma and to add more data to the literature given there are only a handful of reported cases. Our patient is unique in the sense that he was discovered to have renal cell carcinoma shortly after being diagnosed with scleroderma suggesting a paraneoplastic etiology yet continued to worsen despite full resection of the renal cell carcinoma. This is in contrast to the other reported cases of renal cell carcinoma associated scleroderma where scleroderma worsened around the time of the diagnosis of renal cell carcinoma and improved after nephrectomy. There also are case reports of the patients with renal cell carcinoma associated scleroderma where the patient had scleroderma for several years before the diagnosis of renal cell carcinoma, which raises the question, if scleroderma could also represent a risk factor for developing renal cell carcinoma.

Modern approaches to understanding immunopathogenesis of scleroderma (review)

“…В то же время известны случаи, когда диагноз карциномы почек был поставлен через несколько лет после выявления склеродермии. В дальнейшем необходимо решить, может ли склеродермия предрасполагать к развитию карциномы почек [24].…”

Scleroderma as paraneoplastic disease: A new case of a rare association with renal cell carcinoma

“…Dear Editor, We read with interest the case of a 75-year-old male patient diagnosed with renal cell carcinoma (RCC) and systemic sclerosis (SSc) reported by Patel et al 1 SSc can be associated with increased risk of malignancy, most commonly lung, skin and breast cancers, while RCC in SSc is very rare and the relationship between the two diseases is not fully understood. 1 We report an additional case of RCC associated with SSc further supporting the hypothesis that SSc can be a paraneoplastic disease also from RCC.…”