What Is Papillary Renal Cell Carcinoma?
Papillary renal cell carcinoma (PRCC) is a kind of malignant growth that structures inside the coating of the kidney tubules (the tiny cylinders that do the filtration). Generally called "renal cell disease," PRCC is the second most normal sort of malignant kidney growth. The kidneys are a couple of bean-formed organs in the lower back, on both sides of the spine. They are regularly around four and a half inches long. Kidneys are essential for urinary filtration and go about as a "filtration plant" for the circulatory framework. They eliminate the waste and purify the blood.
What Is Hereditary Papillary Cell Carcinoma?
It is a hereditary condition that increases the chance of acquiring cancer of the kidney. It is an inborn condition in which the papillary cells of the kidney can get affected. This condition is known as Type-1 papillary cell cancer. This type of cancer can affect one or both of the kidneys.
What Causes Papillary Cell Cancer of the Kidney?
It is a genetic defect that can be passed from one generation to another. It cannot be predicted if one person will develop this or not. It is unclear why some people may develop this condition whereas others do not. However, there are certain predispositions that may lead to this condition. Habits such as smoking and alcohol can increase one’s chance of acquiring this condition. The risk for type-1 or hereditary conditions is passed through generations. It is believed that mutation of some genes acts as a genetic predisposition. The mutation of the MET (mesenchymal-epithelial transition factor) is believed to be the cause of the hereditary condition. This MET factor prevents excessive cellular multiplication hence preventing cancer-like growth. Due to the absence or mutation of this factor, the cells multiply without any control, hence leading to cancer.
How Is Papillary Carcinoma of the Kidney Inherited?
According to the law of genetics, an embryo receives one set of chromosomes from the father and another set from the mother. Similarly, each and every cell has one genetic copy from each parent. Papillary renal cell carcinoma is an autosomal dominant condition. By being autosomal dominant, it implies that one set of altered or mutated genes is enough to cause the disease. If the parent has the mutated gene, then it can be assumed that the offspring has at least a 50 percent chance of acquiring the condition. This, put together with other risk factors, increases the chance of the occurrence of the disease.
How Common Is Papillary Cell Cancer of the Kidney?
Hereditary papillary renal cell carcinoma is a very rare condition and is known to occur in only one out of millions of people and families.
What Can Be the Symptoms of Papillary Cell Cancer of the Kidney?
Some of the most common symptoms of papillary cell cancer include:
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Pain in the Back: The person may complain of pain in the lower back. This could be intermittent or present all the time. This pain could be unbearable for the patient.
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Blood in the Urine: The person may complain of blood in the urine. This may be present once in a while.
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Sudden Weight Loss: The person may present with sudden and unexplained weight loss.
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Excessive Fatigue: The person may complain of extreme fatigue. Even small exertions can make the person unable to work.
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Constant Low-Grade Fever: The patient may present with a persistent low-grade fever. This could also be due to continuous low-grade infections of the body.
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Swelling at the Back: The patient may have swelling in the lower back. This could be due to a tumor of the kidney.
These symptoms however do not confirm the presence of any cancer. If any of these symptoms occur, then a medical opinion should be obtained at the earliest.
How Will Hereditary Papillary Cancer of the Kidney Be Diagnosed?
If the doctor is suspecting any ailments he may ask to take some tests on the kidneys which may include:
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Analysis of the Urine: The doctor may advise analysis of the urine. This will give an image of the salts of the urine such as urea and creatinine. Any abnormalities in the kidney can cause fluctuations in the levels of these salts.
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Routine Blood Investigation: The doctor may advise a routine blood investigation. This helps assess the overall bodily functions.
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Ultrasound: The doctor may advise an ultrasound to assess the functioning of the kidneys. The presence of kidney stones can be ruled out using this diagnostic test.
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Computed Tomography (CT) Scan: The doctor may suggest a CT scan to check for the presence of any tumors. It can assess the tumor size. Sometimes a special dye can be injected that can give a better contrast image. This dye will be injected into the patient's vein.
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Magnetic Resonance Imaging (MRI) Scan: The MRI Scan can be used in conjunction with the CT scan to arrive at a conclusion. The presence of any tumor can be assessed using this method.
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Genetic Testing: Genetic testing can be performed to assess the occurrence of hereditary cases of this condition. Genetic testing can assess the mutations of the MET gene. It can also be used to test a single gene or a larger panel of genes.
How to Treat Papillary Cancer of the Kidney?
The treatment of the condition depends on the stage of the cancer. The treatment can be a combination of multiple treatment options in order to achieve optimal results. The various treatment modalities can include:
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Surgery: The doctor may remove the tumor surgically. At times the lymph nodes and the adrenal glands may also be removed along with the tumor.
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Radiation Therapy: Certain radiations such as gamma rays can be used to kill the excessively proliferating cells. These radiations can be delivered by external machines or by using radioactive medications.
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Chemotherapy: Chemotherapy can be used to kill rapidly dividing cells. Chemotherapy agents can be delivered using injections or can be given in the form of tablets.
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Bio-Therapy: Bio-Therapy uses the patient's immune system to fight against the dividing cells. This is achieved by developing one’s immunity.
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Targeted Therapy: This is a highly specific treatment that targets only the affected cell and kills them. This causes minimal to no harm to the other cells.
Conclusion
Hereditary papillary renal cancer is a very rare condition in the body. If there is a strong family history of the occurrence of this condition, the physician should be consulted right from the early stages of life. If any of the family members is detected with this condition, it is better to get the other family members genetically analyzed. If any of the aforementioned symptoms are observed, medical care should be given at the earliest. The prognosis depends on the stage of detection. On any day early care gives the best results.
