Renal medullary carcinoma or RMC is a rare and aggressive form of renal carcinoma or kidney cancer where the proliferation of cancer cells usually ensues in the medullary region of the kidney. It is usually noticed in younger individuals of African descent and has garnered attention for its distinctive characteristics and its association with sickle cell trait or sickle cell disease. Early detection and a multidisciplinary approach to treatment are essential for improving outcomes.
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Causes
The exact cause of Renal Medullary Carcinoma remains unclear as in the case of other forms of cancer. However, some studies show that RMC occurs when red blood cells form a sickle shape in the renal medulla, a region of the kidney. The creation of the sickle shape has the potential to cut off the blood flow within the kidney.
This can damage the INI1 or SMARCB1 gene in the renal medulla cells and cause RMC. When researchers examine RMC tumours, they see that this gene is lacking in the chromosome. So they usually check for INI1 gene to determine whether a tumour is RMC or another sort of tumour.
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Risk Factors
Certain causative factors that increase the risk of RMC include:
Sickle Cell Trait or Disease: Individuals with sickle cell trait (a carrier of one abnormal haemoglobin gene) or sickle cell disease (two abnormal haemoglobin genes) are at an increased risk of developing RMC.
Age and Gender: RMC tends to affect adolescents and young adults, particularly males, more often than other age groups and genders.
Symptoms
Recognizing the symptoms of RMC is crucial for early diagnosis and intervention. Common characteristic symptoms of RMC include:
- Blood in urine
- Persistent or severe pain in the side of the abdomen or lower back
- Unexplained and rapid weight loss
- Fatigue and weakness
- Appearance of blood in the urine
- Formation of a mass in the stomach area
- Fevers
- Night sweats
Diagnosis And Treatment
If you detect any of the above-mentioned indications, see a doctor straight away to get diagnosed at the earliest. The doctor typically performs a comprehensive physical examination, recognizes the patient's previous medical history, and then performs a few diagnostic tests, which may include:
Imaging Tests: Imaging studies like CT scans, MRI scans, and ultrasounds are essential to visualize the kidney and detect any abnormalities or tumours
Blood and Urine Tests: Blood tests may be conducted to assess kidney function and check for anaemia. Urine tests can reveal the presence of blood or abnormal cells.
Biopsy: A biopsy, where a small tissue sample is taken for examination, is the definitive way to confirm RMC. However, this procedure is often challenging due to the location of the tumour and the risk of complications.
Treatment
The treatment of RMC typically involves a multimodal approach, considering the cancer's aggressiveness, which is mainly determined by the size of the tumour, the specific location of the disease, and the stage of the malignancy. The latest treatment options for RMC include:
Surgery: Surgical removal of the affected kidney, known as nephrectomy, is often the primary treatment. In some cases, lymph node dissection may be performed.
Chemotherapy: Chemotherapy is administered to target cancer cells that may have spread beyond the kidney.
Radiation Therapy: Radiation therapy may be used to treat RMC, particularly when the cancer has spread to nearby tissues or lymph nodes.
Targeted Therapy: Some targeted therapies and experimental treatments may be considered, but their effectiveness varies.
Clinical Trials: Participation in clinical trials may offer access to innovative treatments and therapies being studied for RMC.
Supportive Care: Supportive care, including pain management and addressing side effects of treatment, is crucial for improving the patient's quality of life.
