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Rare patients in routine care: Treatment and outcome in advanced papillary renal cell carcinoma in the prospective German clinical RCC-Registry
Authors:Michael Staehler  Peter J. Goebell  Lothar Müller  Till-Oliver Emde  Natalie Wetzel  Lisa Kruggel  Martina Jänicke  Norbert Marschner  the RCC-Registry Group (Tumour Registry of Advanced Renal Cell Carcinoma)
Affiliation:1. Department of Urology, Ludwig-Maximilians-University of Munich, University Hospital Campus Grosshadern, Munich, Germany;2. Ambulatory Uro-Oncological Therapy Unit Erlangen (AURONTE), Department of Urology l and Clinic for Haematology and Internistic Oncology, University Hospital Erlangen, Erlangen, Germany;3. Oncology Outpatient-Centre Unter-Ems, Leer, Germany;4. Outpatient-Centre and Day-Hospital for Internistic Oncology and Haematology, Recklinghausen, Germany;5. Clinical Epidemiology and Health Economics, iOMEDICO, Freiburg, Germany;6. Outpatient-Centre for Interdisciplinary Oncology and Haematology, Freiburg, Germany
Abstract:Non-clear cell renal cell carcinoma is a very rare malignancy that includes several histological subtypes. Each subtype may need to be addressed separately regarding prognosis and treatment; however, no Phase III clinical trial data exist. Thus, treatment recommendations for patients with non-clear cell metastatic RCC (mRCC) remain unclear. We present first prospective data on choice of first- and second-line treatment in routine practice and outcome of patients with papillary mRCC. From the prospective German clinical cohort study (RCC-Registry), 99 patients with papillary mRCC treated with systemic first-line therapy between December 2007 and May 2017 were included. Prospectively enrolled patients who had started first-line treatment until May 15, 2016, were included into the outcome analyses (n = 82). Treatment was similar to therapies used for clear cell mRCC and consisted of tyrosine kinase inhibitors, mechanistic target of rapamycin inhibitors and recently checkpoint inhibitors. Median progression-free survival from start of first-line treatment was 5.4 months (95% confidence interval [CI], 4.1–9.2) and median overall survival was 12.0 months (95% CI, 8.1–20.0). At data cutoff, 73% of the patients died, 6% were still observed, 12% were lost to follow-up, and 9% were alive at the end of the individual 3-year observation period. Despite the lack of prospective Phase III evidence in patients with papillary mRCC, our real-world data reveal effectiveness of systemic clear cell mRCC therapy in papillary mRCC. The prognosis seems to be inferior for papillary compared to clear cell mRCC. Further studies are needed to identify drivers of effectiveness of systemic therapy for papillary mRCC.
Keywords:cohort studies  disease management  kidney neoplasms  outcome assessment  outpatients
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