Renal medullary carcinoma: A national analysis of 159 patients

Abstract

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.

Renal medullary carcinoma masquerading as renal infection: a case report

“…Renal medullary carcinoma is a rare and aggressive tumor and nearly uniformly lethal [1][2][3]. The disease occurs almost exclusively in adolescents and young adults with sickle cell hemoglobinopathies [4].…”

Autologous stem‐cell transplant for metastatic renal medullary carcinoma

“…Renal medullary carcinoma (RMC) is a highly aggressive form of renal cell carcinoma (RCC) almost exclusively associated with sickle cell trait and sickle cell disease . Like other forms of RCC, metastatic RMC portends a poor prognosis with median life expectancy less than 1 year . Further, response of metastatic RMC to novel, targeted agents has been disappointing .…”

“…1 Like other forms of RCC, metastatic RMC portends a poor prognosis with median life expectancy less than 1 year. 2,3 Further, response of metastatic RMC to novel, targeted agents has been disappointing. 4 The use of autologous stem cell transplant (ASCT), however, has not been adequately described in the treatment of RMC.…”

Metastatic renal medullary carcinoma: response to chemotherapy and unusual long survival

“…More recently, Ezekian et al [11] reviewed the United States National Cancer Database and identified 159 cases of RMC diagnosed between 1998 and 2011. Median age was 24 (20-31) years.…”