Renal Medullary Carcinoma FAQ

August 8, 2016 / devchris /

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Renal Medullary Carcinoma FAQ

WHAT DOES RMC STAND FOR?

The term “RMC” stands for Renal Medullary Carcinoma.

WHAT IS RENAL MEDULLARY CARCINOMA?

Renal Medullary Carcinoma (RMC) is a rare and aggressive cancer that attacks the kidney. This cancer has a high mortality rate because it quickly spreads to other organs, often before it is diagnosed. Less than 5% of patients will live beyond three years after being diagnosed with RMC. This cancer is found almost exclusively in young patients of African-American descent with sickle cell trait. These individuals may have symptoms of gross hematuria (blood in urine), belly pain mainly in the flanks, unexplained weight loss, difficulty breathing or cough. 

WHAT IS SICKLE CELL TRAIT (SCT)?

Sickle cell trait (SCT) is a blood disorder that causes the body to produce sickle shaped red blood cells. If an individual has SCT, it means that he or she has inherited one sickle cell gene from one parent and one normal gene from the other parent. SCT can never become sickle cell disease, however it is possible for individuals with SCT to pass the gene to their children. In general, people with SCT enjoy normal life spans with no medical problems related to SCT. 

Occasionally people with SCT can have blood in their urine. Under extreme conditions such as high altitude, severe dehydration, or very high intensity physical activity, the red cells can become deformed or sickled and can lead to serious health issues including sudden death.  Other complications include muscle breakdown, reduced blood supply to the spleen, or increased pressure in the eye following eye injuries.  Finally, a very rare form of kidney cancer (renal medullary carcinoma) has been associated with sickle cell trait.

Approximately 7-10% of the African-American population has SCT, and it is also present in people from Latin America, Asia, India, and the Mediterranean. More than 3 million people in the United States and approximately 300 million people worldwide have SCT.

Although testing newborns for the sickle cell gene has been routine in most states since the late 1970’s, many adults in their mid-20’s and older do not know their status.

HOW DO YOU TREAT RENAL MEDULLARY CARCINOMA?

Patients with RMC may be treated with chemotherapy (most common therapy for RMC), surgery (safe and helpful only in a few patients), and radiation therapy (most often used to control symptoms from the disease). There are ongoing clinical trials to find new therapies for RMC.

The at-risk population for RMC is identified at a young age due to the mandate in the United States requiring sickle cell screening to be included in the newborn testing.  It would be beneficial for the at-risk individuals (beginning at infancy) receive annual urine test for blood.  If the test is positive for blood, the need for further evaluation, including imaging studies to rule out RMC must be done. Heightened awareness and early diagnosis along with further research could significantly improve outcomes, thus increasing the overall survival rate.

RESEARCH

Researchers continue to explore the molecular drivers of this rare disease, but few clinical correlations have been made.  As the scientist understand the biological mechanisms of RMC; it is expected that new research opportunities will emerge and new treatment options will be developed for this rare and dismal disease. Currently MD Anderson Cancer Center in Houston, Texas has two active clinical trials specifically designed for patients with RMC and renal cell carcinoma unclassified with medullary phenotype (RMC in patients without sickle cell trait or disease).

Our quest for a CURE begins with AWARENESS and RESEARCH