Wilms tumor is a malignant kidney tumor seen in children and constitutes 95% of childhood kidney tumors. The downside of Wilms tumors is that they grow quickly and tend to spread (metastasize) early.
The incidence?
It is usually seen in children under 5 years of age. It reaches its peak at around 3 years of age. About 16% of the patients are babies in the breastfeeding period. This disease is seen at the same rate in girls and boys. On the other hand, Wilms tumors are also seen in older children and teenagers. Its incidence is one in 10,000. It is seen in both kidneys when diagnosed in 5%.
Reasons?
The causes of Wilms tumor are currently unknown. However, it is known that changes in certain genes and chromosomes contribute to the development of this disease.
What are the symptoms?
Swelling and mass sensation in the abdomen is the complaint in 90% of the cases. The mass is usually hard, painless and smooth. Blood in the urine, high blood pressure, weight loss, sudden onset of abdominal pain by rupture of the tumor, and varicocele in the testicle on that side are rare symptoms.
Congenital anomalies (undescended testis, hypospadias, kidney fusion anomalies) are also present in 10% of patients. Wilms tumor occurs more frequently with some other developmental formations and/or hereditary cancer syndromes compared to other tumors seen in childhood. Wilms tumor is a component in WAGR (Wilms tumor, aniridia, genitourinary anomaly, mental retardation) and Denys-Drash (DDS) syndromes. The risk of developing Wilms tumor is increased in Beckwith-Wiedemann (BWS), Pearl-man and Simpson-Golabi-Behmel syndromes.
How is the diagnosis made?
The first step in imaging is ultrasound to differentiate the location and content of the mass (solid/cystic). For surgical planning, computed tomography or magnetic resonance imaging (MR) is performed by administering contrast material orally and intravenously. With echocardiography, it should be checked whether the clot formed by the tumor in the vessel extends to the heart. Lung tomography is also requested for the evaluation of lung metastases.
A biopsy may be required for definitive diagnosis in cases where the definitive diagnosis is not fully established, in suspected cases or in cases with insufficient response to drug therapy.
How is it treated?
The treatment of a patient with Wilms tumor is first surgery and then chemotherapy. It is removed in the kidney along with the tumor in the surgery. The less the disease has spread at the time of initial diagnosis, the higher the response to treatment will be.
In advanced diseases, the process may be a little more challenging as it will be more difficult to control the disease. The treatment plan is made according to the size of the tumor and its prevalence in the body. Treatment usually begins with chemotherapy. The purpose of this is to shrink the tumor and bring it to a size that can be operated more easily. After the surgery, additional irradiation of the tumor area (radiotherapy) may be required depending on the tumor stage and/or the presence of metastases.
What are the chances of success?
Wilms tumor is one of the cancer types that has shown the most improvement in treatment success in recent years. If the tumor is detected at an early stage and has good characteristics, the chance of recovery is around 90% in these cases. With recent developments, the chance of survival is high even in advanced disease.
If the disease recurs (in cases of recurrence), new drug treatments, additional dose radiotherapy and surgery can be performed by evaluating the treatments given.