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Shah AY, Karam JA, Malouf GG, Rao P, Lim ZD, Jonasch E, Xiao L, Gao J, Vaishampayan UN, Heng DY, Plimack ER, Guancial EA, Fung C, Lowas SR, Tamboli P, Sircar K, Matin SF, Rathmell WK, Wood CG, Tannir NM
Management and Outcomes of Patients with Renal Medullary Carcinoma: A Multi-Center Collaborative Study
BJU Int (2016) In process.
OBJECTIVE: To describe the management strategies and outcomes of patients with renal medullary carcinoma (RMC) and characterize predictors of overall survival (OS). PATIENTS AND METHODS: RMC is a rare and aggressive malignancy that afflicts young patients with sickle cell trait; there are limited data on management to date. This is a study of patients with RMC who were treated during 2000-2015 at eight academic institutions in North America and France. The Kaplan-Meier method was used to estimate OS, measured from initial RMC diagnosis to date of death. Cox regression analysis was used to determine predictors of OS. RESULTS: Fifty-two patients (37 males) were identified. Median age at diagnosis was 28 years (range 9-48). Forty-nine patients (94%) had stage III/IV. Median OS for all patients was 13.0 months. Thirty-eight patients (75%) had nephrectomy. Patients who underwent nephrectomy had superior OS compared to patients who were treated with systemic therapy only (median OS 16.4 vs. 7.0 months, p=0.0004). Forty-five patients received chemotherapy and 13 (29%) had an objective response; 28 patients received targeted therapies, with 8-week median therapy duration and no objective responses. Only seven patients (13%) survived longer than two years. CONCLUSIONS: RMC carries a poor prognosis. Chemotherapy provides palliation and remains the mainstay of therapy, but less than 20% of patients survive longer than two years, underscoring the need to develop more effective therapy for this rare tumor. In this study, nephrectomy was associated with improved OS. This article is protected by copyright. All rights reserved.
Publication Date: 2016-11-08.
Last updated on Saturday, August 22, 2020