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Renal Medullary Carcinoma - An Overview

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Renal medullary carcinoma is an uncommon kidney malignancy. Read below to know more.

Written by

Dr. Aysha Anwar

Medically reviewed by

Dr. Yash Kathuria

Published At April 17, 2024
Reviewed AtApril 17, 2024

Introduction:

Renal cell carcinoma is a highly aggressive kind of kidney cancer that was first reported in 1995. Within four months of diagnosis, half of the renal medullary carcinoma patients reported in the initial 1995 study died. With current medications, this has improved to 13 months, and efforts are being made to discover new and more effective ones. If discovered early, renal medullary carcinoma may be simpler to cure. Young black people should, therefore, get in touch with their healthcare physician once they develop any symptoms that could indicate renal medullary carcinoma, especially if they are known to have sickle cell disease or the sickle cell trait.

What Is Renal Medullary Carcinoma?

A rare form of kidney cancer called renal medullary carcinoma (renal medullary carcinoma) primarily affects those who have sickle cell trait. Men are twice as likely as women to have renal medullary carcinoma, and the majority of those affected are under 30. Before being detected, renal medullary carcinoma typically originates in the right kidney but can extend to other organs.

Less than five percent of renal medullary carcinoma patients survive for three years after diagnosis. Early diagnosis may make RMC simpler to treat. Renal side pain and blood in the urine are the most typical signs of renal metastatic cancer (RMC). Radiation therapy, surgery, or chemotherapy are frequently used as forms of treatment.

Why Does Renal Medullary Cancer Occur?

  • The reasons behind RMC are yet unknown to everyone. Due to a blood disorder, red blood cells sickle in almost all renal medullary carcinoma patients. This includes sickle cell trait, sickle cell disease, and "sickle hemoglobinopathies." There are no additional recognized renal medullary carcinoma risk factors.

  • Most individuals with sickle cell trait have RMC. This could be because the sickle cell trait is 50 times more common than other sickle hemoglobinopathies. Approximately one in 14 Black individuals have sickle cell disease.

  • INI1, sometimes known as SMARCB1, is a protein absent from tumors in patients with RMC. As a "Tumor Suppressor," this protein keeps healthy cells from developing into cancerous ones. Sickled red blood cells obstruct blood flow to the renal medulla, the kidney's innermost portion. This may harm the gene required for kidney cells to make the INI1 protein.

What Signs of Renal Medullary Cancer Are Present?

If a person encounters renal medullary carcinoma symptoms, consult a physician immediately. Among the most typical symptoms are:

  • Urine containing blood (hematuria).

  • Discomfort near the kidneys.

  • There is a noticeable lump on the right side.

  • Getting in shape.

  • Sweating at night and fever.

  • A timely diagnosis is essential for improving treatment results. RMC cannot currently be prevented or screened for. For this reason, those who have sickle cell disease (SCD) or Sickle cell trait must understand these signs.

How Does One Diagnose Renal Medullary Carcinoma?

  • Early diagnosis of RMC is challenging due to the absence of early symptoms.

  • When sickle cell trait carriers experience renal medullary carcinoma symptoms, they typically have an ultrasonography. This can make the kidneys easier for doctors to see. If a tumor is detected,

  • Additional imaging methods can be used to assess its malignant nature. This comprises Magnetic Resonance Imaging (MRI) and Computed Tomography (CT) scans.

  • Medical professionals will then take a sample if imaging reveals a kidney tumor that might be malignant. This implies that a tiny sample of the kidney tumor tissue will be taken. The tissue will be examined under a microscope, and its INI1 content will be determined. An INI1 deficiency will validate an RMC diagnosis.

What Is the Treatment of Renal Medullary Carcinoma?

Renal medullary carcinoma patients receive the following therapies:

  1. Chemotherapy: Drugs used in chemotherapy eradicate cancer cells, limit their growth, or lessen symptoms associated with the illness. A single medicine or a combination of two or more drugs may be used in chemotherapy, depending on the type of cancer and its growth rate.

  2. Operation: During renal medullary carcinoma surgery, which is also known as radical nephrectomy, the entire kidney is typically removed. This is because RMC tumors are situated deep within the kidney. Furthermore, because the margins of these tumors are hazy, cancerous and healthy cells coexist. Cancer cells can remain if the surgeon aims to remove the tumor alone.

  3. Radiation Treatment: Strong, targeted radiation beams are used in radiation therapy to destroy cancer cells. Radiation therapy is administered using various procedures. These allow physicians to target a tumor precisely with the least harm to surrounding healthy tissue.

IMRT, or intensity-modulated radiation therapy, directs several radiation beams with varying intensities at the tumor to provide the maximum dose. Alternatively referred to as stereotactic ablative radiotherapy or stereotactic ablative body radiation, Stereotactic body radiation therapy. SBRT uses extremely high radiation doses to target malignancies precisely. This is accomplished by directing multiple radiation beams of varying intensity toward the tumor from diverse directions.

  1. Proton Treatment: Like conventional radiation therapy, proton therapy employs a different energy that might let medical professionals target malignancies more precisely. This minimizes radiation damage to adjacent healthy tissue and permits the administration of a stronger radiation dosage.

Is It Possible to Prevent Renal Medullary Carcinoma?

It is yet unknown how to avoid RMC or determine if one has it in the absence of symptoms. Finding out if one has sickle cell hemoglobinopathy and eyeing for signs can be the best option for renal medullary carcinoma.

What Is the Renal Medullary Carcinoma Prognosis?

According to one study, the average survival period is 13 months. Longer life with various therapies has been demonstrated in other trials. Compared to other cancer kinds, there are not as many studies on this particular cancer because it is rare. Connecting disparate situations accounts for a large portion of the knowledge base. Most people's cancers have spread to their lymph nodes or other organs by the time they receive a diagnosis.

Conclusions:

Patients with renal medullary carcinoma may survive longer if a high index of suspicion is maintained. This can also result in earlier diagnosis and treatment. RMC is an extremely uncommon, aggressive cancer that can strike any age. With a median survival of only four months, metastatic disease is nearly always present at the time of diagnosis, contributing to RMC's dismal prognosis. It usually manifests in young African individuals with sickle cell trait or illness, with the right kidney being involved in more than 75 percent of cases.

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Dr. Yash Kathuria
Dr. Yash Kathuria

Family Physician

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