Wilms Tumor
Wilms Tumor Wilms tumor, also called Nephroblastoma, is the most common type of pediatric kidney cancer, accounting for about 5% of all childhood cancers. “It is named after Max Wilms, a German doctor who wrote one of the first medical articles about the disease in 1899” (The American Cancer Society, 2016). Majority of cases are diagnosed before the age of 5, becoming less common as the child grows older. There are approximately 500 new cases diagnosed every year in the United States. During fetal renal development, early kidney cells develop into glomeruli and nephrons. If this process fails, clusters of immature cells may form in the kidneys when the baby is born. These cells will usually mature by the time a child is 3 or 4 years
…show more content…
old, but some may grow out of control creating a mass of immature cells, the Wilms tumor (ASCO, 2016). Wilms tumor usually only affects one kidney, with rare possibility of multiple tumors in the same kidney. Only about 5% of children will have tumors in both kidneys. Based on their histology, Wilms tumors are divided into 2 major groups. The tumors with favorable histology have a good prognosis as they respond better to chemotherapy unlike the ones with unfavorable histology. The unfavorable histology shows signs of anaplasia (loss of structural differentiation in cells), which makes it more difficult to cure. Fortunately, the tumors with favorable histology are much more common, about 9 out of every 10 cases (The American Cancer Society, 2016).
Signs and Symptoms
By the time the first signs and symptoms appear, the tumor has already grown quite large.
The tumor is usually much larger than the kidney itself. Children often do not experience any pain or problems and look perfectly healthy. Fortunately, Wilms tumor is usually found before it metastasis to other parts of the body. The first thing parents most often notice is a hard, palpable mass in the abdominal area. Rarely children present with an abdominal pain. Some other symptoms that can accompany this disease are hematuria, hypertension, anemia, fatigue, fever, nausea, weight loss, shortness of breath and feeling of being …show more content…
tired.
Diagnosis
As with any other diagnosis, the physician will start with complete medical history, family history of cancer and other diseases and physical examination. Blood and urine analysis will also be conducted.
Imaging Tests
If a doctor suspects that the child may have a tumor, he/she will order a variety of diagnostic tests.
Ultrasound
Abdominal ultrasound is generally the first imaging test done. Ultrasound is good way to visualize the abdominal organs without exposing a child to radiation. It can determine whether there is a tumor, the composition of the mass, whether it is cystic or solid. It can also see if it has spread into surrounding organs or if it has invaded major vessels such as the renal vein or inferior vena cava. CT (CAT) scan or MRI scan
“Contrast-enhanced computed tomography (CT) or magnetic resonance imaging (MRI) is recommended to further evaluate the nature and extent of the mass” (Chintagumpala and Muscal, 2016). Both of these techniques produce detailed images of the body by using x-rays in the CT scan and magnetic field in MRI. This gives the physician better idea whether the tumor has spread into the surrounding organs or into the nearby veins. Chest x-ray
Chest x-ray may be ordered to determine if the tumor has metastasized into the lungs. This can also be done by chest CT scan.
Surgery/biopsy
The imaging tests usually give the physician enough confirmation whether the child has a Wilms tumor. In this case, the surgery is usually performed and affected kidney and tumor are removed. If more clarification is needed or when the tumor is very large, biopsy is performed first. A small sample of the tissue is removed and analyzed under a microscope by a pathologist.
Staging
After Wilms tumor is diagnosed, it is important to find out how large the tumor is, how far it has spread and whether it has invaded other tissues. This process is called staging and it is based on the results of the physical exam and imaging tests (ultrasound, CT scans, etc.), as well as on the results of surgery, if it has been done (The American Cancer Society, 2016). There are 5 stages. The higher the number, the more extensive the disease is with worse prognosis. Stage I
The tumor was contained within one kidney. It has not grown into the blood vessels. It was completely removed by surgery and the renal capsule was not broken. There was no biopsy done prior to surgery. Stage I accounts for about 40% of cases (The American Cancer Society, 2016). Stage II
The cancer has spread beyond the kidney but was completely removed during surgery. No biopsy was performed before the surgery. About 20% of cases fall in this group (The American Cancer Society, 2016). Stage III
Cancer has spread into the nearby lymph nodes or organs and cannot be completely removed during the surgery. It is however limited to the abdominal area. About 20% of all Wilms tumors are stage III (The American Cancer Society, 2016). Stage IV
Cancer has metastasized into lungs, liver, brain, or bones and has spread into lymph nodes outside of the abdominal area. Approximately 10% of cases fall in this category (The American Cancer Society, 2016). Stage V
Cancer cells are found in both kidneys. In this case, each kidney is staged separately. Only about 5% of all cases are stage V (The American Cancer Society, 2016).
Treatment
The treatments of Wilms tumor include surgery, chemotherapy, and radiation therapy. The course of treatment depends on many factors such as the type and stage of the tumor as well as the child’s overall health.
Surgery
Surgery is the main treatment for Wilms tumor. The surgeon will either perform a radical nephrectomy or partial nephrectomy. During radical nephrectomy, the entire kidney and some surrounding tissue, as well as nearby lymph nodes are removed. With partial nephrectomy, only the tumor and some surrounding healthy tissue are removed, leaving as much of the kidney as possible (ASCO, 2016). Chemotherapy
“Chemotherapy is the use of drugs to destroy cancer cells, usually by stopping the tumor cells’ ability to grow and divide” (ASCO, 2016). Chemotherapy can be used to shrink a tumor prior to surgery or be given after the surgery to eliminate remaining cancer cells. “The type of chemotherapy drug and the length of therapy depends on tumor histology and stage” (Gregory, 2015). Radiation therapy
Radiation therapy is used to target and destroy cancer cells that may have remained after the surgery. It is generally given to patients diagnosed with an advanced stage of Wilms tumor or those who have tumors with unfavorable histology.
Prognosis
“Overall five-year survival rates for Wilms tumor have steadily improved from 20 percent in the late 1960s to 90 percent in subsequent clinical trials from both National Wilms Tumor Study/Children’s Oncology Group (NWTS/COG) and International Society of Pediatric Oncology (SIOP) groups” (Chintagumpala and Muscal, 2016). The prognosis and survival rate depend of factors such as tumor histology, tumor stage, molecular and genetic markers and age > 2. The prognosis would generally get worse with age. Gregory (2015) has written in his study that the cure rates for lower-stage tumors range from 85-90%. For children with more advanced disease, cure rates range from 60% with unfavorable histology to 90% with favorable histology. However, there is a chance of recurrence, typically within the first 2 years of diagnosis, therefore the follow up care is extremely important.
old, but some may grow out of control creating a mass of immature cells, the Wilms tumor (ASCO, 2016). Wilms tumor usually only affects one kidney, with rare possibility of multiple tumors in the same kidney. Only about 5% of children will have tumors in both kidneys. Based on their histology, Wilms tumors are divided into 2 major groups. The tumors with favorable histology have a good prognosis as they respond better to chemotherapy unlike the ones with unfavorable histology. The unfavorable histology shows signs of anaplasia (loss of structural differentiation in cells), which makes it more difficult to cure. Fortunately, the tumors with favorable histology are much more common, about 9 out of every 10 cases (The American Cancer Society, 2016).
Signs and Symptoms
By the time the first signs and symptoms appear, the tumor has already grown quite large.
The tumor is usually much larger than the kidney itself. Children often do not experience any pain or problems and look perfectly healthy. Fortunately, Wilms tumor is usually found before it metastasis to other parts of the body. The first thing parents most often notice is a hard, palpable mass in the abdominal area. Rarely children present with an abdominal pain. Some other symptoms that can accompany this disease are hematuria, hypertension, anemia, fatigue, fever, nausea, weight loss, shortness of breath and feeling of being …show more content…
tired.
Diagnosis
As with any other diagnosis, the physician will start with complete medical history, family history of cancer and other diseases and physical examination. Blood and urine analysis will also be conducted.
Imaging Tests
If a doctor suspects that the child may have a tumor, he/she will order a variety of diagnostic tests.
Ultrasound
Abdominal ultrasound is generally the first imaging test done. Ultrasound is good way to visualize the abdominal organs without exposing a child to radiation. It can determine whether there is a tumor, the composition of the mass, whether it is cystic or solid. It can also see if it has spread into surrounding organs or if it has invaded major vessels such as the renal vein or inferior vena cava. CT (CAT) scan or MRI scan
“Contrast-enhanced computed tomography (CT) or magnetic resonance imaging (MRI) is recommended to further evaluate the nature and extent of the mass” (Chintagumpala and Muscal, 2016). Both of these techniques produce detailed images of the body by using x-rays in the CT scan and magnetic field in MRI. This gives the physician better idea whether the tumor has spread into the surrounding organs or into the nearby veins. Chest x-ray
Chest x-ray may be ordered to determine if the tumor has metastasized into the lungs. This can also be done by chest CT scan.
Surgery/biopsy
The imaging tests usually give the physician enough confirmation whether the child has a Wilms tumor. In this case, the surgery is usually performed and affected kidney and tumor are removed. If more clarification is needed or when the tumor is very large, biopsy is performed first. A small sample of the tissue is removed and analyzed under a microscope by a pathologist.
Staging
After Wilms tumor is diagnosed, it is important to find out how large the tumor is, how far it has spread and whether it has invaded other tissues. This process is called staging and it is based on the results of the physical exam and imaging tests (ultrasound, CT scans, etc.), as well as on the results of surgery, if it has been done (The American Cancer Society, 2016). There are 5 stages. The higher the number, the more extensive the disease is with worse prognosis. Stage I
The tumor was contained within one kidney. It has not grown into the blood vessels. It was completely removed by surgery and the renal capsule was not broken. There was no biopsy done prior to surgery. Stage I accounts for about 40% of cases (The American Cancer Society, 2016). Stage II
The cancer has spread beyond the kidney but was completely removed during surgery. No biopsy was performed before the surgery. About 20% of cases fall in this group (The American Cancer Society, 2016). Stage III
Cancer has spread into the nearby lymph nodes or organs and cannot be completely removed during the surgery. It is however limited to the abdominal area. About 20% of all Wilms tumors are stage III (The American Cancer Society, 2016). Stage IV
Cancer has metastasized into lungs, liver, brain, or bones and has spread into lymph nodes outside of the abdominal area. Approximately 10% of cases fall in this category (The American Cancer Society, 2016). Stage V
Cancer cells are found in both kidneys. In this case, each kidney is staged separately. Only about 5% of all cases are stage V (The American Cancer Society, 2016).
Treatment
The treatments of Wilms tumor include surgery, chemotherapy, and radiation therapy. The course of treatment depends on many factors such as the type and stage of the tumor as well as the child’s overall health.
Surgery
Surgery is the main treatment for Wilms tumor. The surgeon will either perform a radical nephrectomy or partial nephrectomy. During radical nephrectomy, the entire kidney and some surrounding tissue, as well as nearby lymph nodes are removed. With partial nephrectomy, only the tumor and some surrounding healthy tissue are removed, leaving as much of the kidney as possible (ASCO, 2016). Chemotherapy
“Chemotherapy is the use of drugs to destroy cancer cells, usually by stopping the tumor cells’ ability to grow and divide” (ASCO, 2016). Chemotherapy can be used to shrink a tumor prior to surgery or be given after the surgery to eliminate remaining cancer cells. “The type of chemotherapy drug and the length of therapy depends on tumor histology and stage” (Gregory, 2015). Radiation therapy
Radiation therapy is used to target and destroy cancer cells that may have remained after the surgery. It is generally given to patients diagnosed with an advanced stage of Wilms tumor or those who have tumors with unfavorable histology.
Prognosis
“Overall five-year survival rates for Wilms tumor have steadily improved from 20 percent in the late 1960s to 90 percent in subsequent clinical trials from both National Wilms Tumor Study/Children’s Oncology Group (NWTS/COG) and International Society of Pediatric Oncology (SIOP) groups” (Chintagumpala and Muscal, 2016). The prognosis and survival rate depend of factors such as tumor histology, tumor stage, molecular and genetic markers and age > 2. The prognosis would generally get worse with age. Gregory (2015) has written in his study that the cure rates for lower-stage tumors range from 85-90%. For children with more advanced disease, cure rates range from 60% with unfavorable histology to 90% with favorable histology. However, there is a chance of recurrence, typically within the first 2 years of diagnosis, therefore the follow up care is extremely important.