BACKGROUND: Renal medullary carcinoma (RMC) is an aggressive malignancy seen predominantly in young males with sickle cell trait. RMC is poorly understood, with fewer than 220 cases described in the medical literature to date. We used a large national registry to define the typical presentation, treatments, and outcomes of this rare tumor. METHODS: The National Cancer Database was queried for patients under 40 years of age diagnosed with RMC from 1998 to 2011. An analysis of patient and tumor characteristics, treatment details, and overall survival (OS) was undertaken, and factors associated with mortality were identified using multivariable regression analysis. RESULTS: In total, 159 patients with RMC were identified, of whom a majority were male (71%), African American (87%), and had metastatic disease (71%). Median tumor size was 6 cm and median survival was 7.7 months. Most patients underwent surgery (60%) and chemotherapy (65%). Few patients received radiation (12%). Patients with metastatic disease had a significantly worse median survival (4.7 vs. 17.8 months, P < 0.001) and were less likely to receive surgery (42% vs. 91%, P < 0.001). Age and tumor size did not appear to impact OS. CONCLUSION: In the largest cohort to date of patients with RMC, we found a dismal median survival of less than 8 months. Age and tumor size were not associated with OS. Metastatic disease at presentation was the main negative prognostic indicator in RMC and was present in a majority of patients at the time of diagnosis.
BACKGROUND:Renal medullary carcinoma (RMC) is an aggressive malignancy seen predominantly in young males with sickle cell trait. RMC is poorly understood, with fewer than 220 cases described in the medical literature to date. We used a large national registry to define the typical presentation, treatments, and outcomes of this rare tumor. METHODS: The National Cancer Database was queried for patients under 40 years of age diagnosed with RMC from 1998 to 2011. An analysis of patient and tumor characteristics, treatment details, and overall survival (OS) was undertaken, and factors associated with mortality were identified using multivariable regression analysis. RESULTS: In total, 159 patients with RMC were identified, of whom a majority were male (71%), African American (87%), and had metastatic disease (71%). Median tumor size was 6 cm and median survival was 7.7 months. Most patients underwent surgery (60%) and chemotherapy (65%). Few patients received radiation (12%). Patients with metastatic disease had a significantly worse median survival (4.7 vs. 17.8 months, P < 0.001) and were less likely to receive surgery (42% vs. 91%, P < 0.001). Age and tumor size did not appear to impact OS. CONCLUSION: In the largest cohort to date of patients with RMC, we found a dismal median survival of less than 8 months. Age and tumor size were not associated with OS. Metastatic disease at presentation was the main negative prognostic indicator in RMC and was present in a majority of patients at the time of diagnosis.
Authors: Alessandro Carugo; Rosalba Minelli; Luigi Sapio; Melinda Soeung; Federica Carbone; Frederick S Robinson; James Tepper; Ziheng Chen; Sara Lovisa; Maria Svelto; Samirkumar Amin; Sanjana Srinivasan; Edoardo Del Poggetto; Sara Loponte; Francesca Puca; Prasenjit Dey; Gabriel G Malouf; Xiaoping Su; Liren Li; Dolores Lopez-Terrada; Dinesh Rakheja; Alexander J Lazar; George J Netto; Priya Rao; Alessandro Sgambato; Anirban Maitra; Durga N Tripathi; Cheryl L Walker; Jose A Karam; Timothy P Heffernan; Andrea Viale; Charles W M Roberts; Pavlos Msaouel; Nizar M Tannir; Giulio F Draetta; Giannicola Genovese Journal: Cancer Cell Date: 2019-02-11 Impact factor: 38.585
Authors: Darmood Wei; Youfeng Yang; Christopher J Ricketts; Cathy D Vocke; Mark W Ball; Carole Sourbier; Darawalee Wangsa; Danny Wangsa; Rajarshi Guha; Xiaohu Zhang; Kelli Wilson; Lu Chen; Paul S Meltzer; Thomas Ried; Craig J Thomas; Maria J Merino; W Marston Linehan Journal: Genes Chromosomes Cancer Date: 2020-04-17 Impact factor: 5.006
Authors: Andrew L Hong; Yuen-Yi Tseng; Jeremiah A Wala; Won-Jun Kim; Bryan D Kynnap; Mihir B Doshi; Guillaume Kugener; Gabriel J Sandoval; Thomas P Howard; Ji Li; Xiaoping Yang; Michelle Tillgren; Mahmhoud Ghandi; Abeer Sayeed; Rebecca Deasy; Abigail Ward; Brian McSteen; Katherine M Labella; Paula Keskula; Adam Tracy; Cora Connor; Catherine M Clinton; Alanna J Church; Brian D Crompton; Katherine A Janeway; Barbara Van Hare; David Sandak; Ole Gjoerup; Pratiti Bandopadhayay; Paul A Clemons; Stuart L Schreiber; David E Root; Prafulla C Gokhale; Susan N Chi; Elizabeth A Mullen; Charles Wm Roberts; Cigall Kadoch; Rameen Beroukhim; Keith L Ligon; Jesse S Boehm; William C Hahn Journal: Elife Date: 2019-03-12 Impact factor: 8.140
Authors: William C Hahn; Matthew Meyerson; Andrew L Hong; Kar-Tong Tan; Hyunji Kim; Jian Carrot-Zhang; Yuxiang Zhang; Won Jun Kim; Guillaume Kugener; Jeremiah A Wala; Thomas P Howard; Yueh-Yun Chi; Rameen Beroukhim; Heng Li; Gavin Ha; Seth L Alper; Elizabeth J Perlman; Elizabeth A Mullen Journal: Genome Med Date: 2021-07-14 Impact factor: 11.117