Wilm’s tumor also known as nephroblastoma occurs due to the mutation in the WT gene (Wilms Tumor 1 & 2 genes). It is most commonly seen in children less than six years of age with a median age of about 3 years.
Is Wilm’s Tumor A Serious Condition?
It is a malignant tumor of kidneys which means it can metastasize that is it can spread to other parts of the body which makes it a very serious condition and requires immediate treatment. Delay of the treatment can result in life-threatening consequences and even fatal to your child.
It also has few syndromic associations with it like Beckwith Weidman syndrome, WAGR syndrome, Denys Drash syndrome, Edward syndrome which basically means that it can also occur adjacent to other diseases and can be more dangerous than occurring alone.
It is a malignant tumor of the kidneys metastasizing especially in the lungs. It occurs at the upper pole of the kidneys and can be unilateral or bilateral. It is a very large tumor presents as an abdominal mass on palpation. It can present as abdominal pain, fever and can be associated with hematuria (i.e. blood in urine), hypertension, difficulty in urination, respiratory symptoms like cough, etc. Various investigations like CT scan, MRI, abdominal ultrasound, a renal biopsy can confirm the diagnosis of Wilm’s tumor. It is a radio and chemotherapy responsive tumor. (1)
Can Wilm’s Tumor Be Reversed?
Although, being malignant many tumors are not curable but this tumor has a specialty that even being a malignant tumor it can be treated successfully in more than 90% cases and has very good prognosis which means that patient can live a healthy life after successful treatment. It also has a very low recurrence rate.
The survival rate in children suffering from it is about 90% and even above sometimes when detected in early stages like stage 1 or stage 2.
The best treatment available and also the treatment of choice is surgery which consists of removal of the kidney affected known as nephrectomy that is the removal of the affected kidney. It may be unilateral or bilateral depending upon the involvement of the kidneys in the tumor. If kidney involvement is bilateral then bilateral nephrectomy is usually associated with renal failure. In unilateral nephrectomy the other kidney which is unaffected can take over the function of both kidneys and patient can live a normal and healthy life without any complication whereas in bilateral nephrectomy patient has to be kept either on dialysis or a renal transplant is required so as to perform the function of kidneys in the body.
Medical Treatment: Various anticancer drugs are used in the treatment of Wilm’s tumor. The treatment regimen followed is based on the staging of the tumor and is divided into 2 types.
- Regimen 1: Dactinomycin, Vincristine, Adriamycin(doxorubicin)
- Regimen 2: Regime 1 drugs plus cyclophosphamide and etoposide
Folinic acid tablets and iron therapy can be given for the fast recovery of the normal cells from the anticancer drugs. Chemotherapy is started just after the nephrectomy to prevent further spreading of cancer in the body. If there is bilateral involvement of the kidneys then only chemotherapy can be given for two years. Continuous monitoring is required with the help of an abdominal ultrasound and CT scan to check for the metastasis and the staging of the tumor.
Radiotherapy: If the tumor is large or in the advanced stage then radiotherapy has also to be given. It increases the survival rate from 50% to 80% even in most advanced cases of the tumor which implies its high curability and reversal rate. (2)
Although it is an anaplastic and malignant tumor with high malignant potential to lungs and other body parts it has a high reversibility rate. The prognosis stands at 90% reversibility with appropriate surgical and medical treatment which is a very relieving factor for a malignant tumor. Early detection and treatment are the keys to the success of the cure of Wilm’s tumor.
- Breslow N, Olshan A, Beckwith JB, Green DM. Epidemiology of Wilms tumor. Med Pediatr Oncol. 1993. 21(3):172-81. [Medline].
- Paulino AC, Wilimas J, Marina N, et al. Local control in synchronous bilateral Wilms tumor. Int J Radiat Oncol Biol Phys. 1996 Oct 1. 36(3):541-8. [Medline].
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