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• W2230555292 abstract "ABSTRACT Aim: Renal medullary carcinoma (RMC) is a rare, highly aggressive renal neoplasm. In absence of prospective trials, a systematic review of RMC publications was performed in order to characterize clinical features and treatment. Methods: We found 68 articles on RMC in Pubmed from 1987 to January 2014 (key words “Carcinoma, renal cell” OR “kidney neoplasm” AND “medullary”). Results: 123/135 cases were analysed (12 missing data). Median age was 21.9y (10-69). Male/female ratio was 2:1. Afro-Americans represented 83% of pts, Caucasians, Arabs / Brazilians were 8.7% and 2.6% respectively. Cases related to sickle cell trait accounted 89% while homozygous form and hemoglobinopathy free status were 8% and 3% respectively. Common initial symptoms were hematuria (49%), flank pain (55%) and weight loss (14%). Time from symptoms to diagnosis had a mean of 4.7 m. At diagnosis, only 2% had localized tumor while 98% were metastatic: secondary lymph node involvement (62.5%), lung (48%), liver (33%), bone (32%), pleura (16%) and brain (16%). Right kidney was involved in 74% of cases. Survival data was available in 82 pts (66%). Ten pts (12%) were alive at the moment of publication. Median overall survival was 9 m (0-92). Patients with visceral metastasis (96%) had the worst mean survival (6.3m) while pts with isolated-lymph-node involvement (12%) showed better outcome (OS = 14m). No responses were detected with immunotherapy (OS = 3m). Best outcomes were displayed with chemotherapy combination regimens based on platinum derivatives, gemcitabine, paclitaxel (n = 9) or based on MVAC (n = 14), with median survivals of 10 and 15 months respectively. High-dose chemotherapy followed by autologous stem cell transplantation seems useful but not sufficiently explored (n = 1, OS = 22m). Anecdotal cases of targeted therapy included: Bortezomib (n = 2, OS= 27 m and 6 m), Thalidomide (n = 2, OS = 52 m and 2 m), Sunitinib (n = 10, OS = 2 m), Imatinib (n = 1, OS = 1m). Loss of immunoexpression and gene inactivation of SMARCB1/INI were the only molecular alterations constantly found (n = 28/28). Conclusions: RMC, a rare subtype of renal cell carcinoma with poor outcome, mainly affects a particular population of young afro-Americans with hemoglobinopathy. Better biological understanding and molecular characterization of RMC will facilitate improving the treatment and survival of these patients. Disclosure: All authors have declared no conflicts of interest." @default.
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• W2230555292 date "2014-09-01" @default.
• W2230555292 modified "2023-09-27" @default.
• W2230555292 title "Renal Medullary Carcinoma: a Systematic Review" @default.
• W2230555292 doi "https://doi.org/10.1093/annonc/mdu337.27" @default.
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