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Modeling Spontaneous Metastatic Renal Cell Carcinoma mRCC in Mice Following Nephrectomy
Published on: April 29, 2014
Renal medullary carcinoma: A national analysis of 159 patients
Brian Ezekian1, Brian Englum1, Brian F Gilmore1
1Department of Surgery, Duke University Medical Center, Durham, North Carolina.
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.
Insights
Renal medullary carcinoma (RMC) has a dismal median survival under 8 months. Metastatic disease at diagnosis is the primary negative prognostic factor for this rare cancer.
Area of Science:
- Oncology
- Rare Cancers
- Renal Cell Carcinoma
Background:
- Renal medullary carcinoma (RMC) is an aggressive cancer predominantly affecting young males with sickle cell trait.
- Fewer than 220 cases of RMC have been reported in medical literature, highlighting its rarity.
- Understanding RMC presentation, treatment, and outcomes is crucial due to its poor prognosis.
Purpose of the Study:
- To define the typical presentation, treatments, and outcomes of RMC.
- To identify factors associated with mortality in RMC patients.
- To analyze the largest cohort of RMC patients to date.
Main Methods:
- Utilized the National Cancer Database to identify RMC patients under 40 diagnosed between 1998 and 2011.
- Analyzed patient and tumor characteristics, treatment details, and overall survival (OS).
- Employed multivariable regression analysis to identify mortality predictors.
Main Results:
- Identified 159 RMC patients: 71% male, 87% African American, 71% with metastatic disease.
- Median survival was 7.7 months; patients with metastatic disease had significantly worse survival (4.7 vs. 17.8 months).
- Metastatic disease at presentation was linked to lower surgical rates (42% vs. 91%) and worse survival; age and tumor size did not impact OS.
Conclusions:
- The largest RMC cohort reveals a dismal median survival of less than 8 months.
- Metastatic disease at diagnosis is the primary negative prognostic indicator for RMC.
- Age and tumor size were not associated with overall survival in RMC patients.
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