1229 Exam 7: Childhood Cancers Flashcards
Wilms’ Tumor
- The most common intra-abdominal and kidney tumor of childhood
- It is believed to arise from cells that mutate or fail to develop properly in the womb
- Usually evident by the time the child is 3 or 4; rarely occurs after age 6
- Also called a Nephroblastoma
- Occurrence is associated with a genetic inheritance and with several congenital anomalies
- Exact cause is unknown
Risk Factors for Wilms’ Tumor
- Girls are slightly more likely to develop Wilms’ tumor than boys
- Black children have a slightly higher risk; Asian-Americans appear to have a lower risk
- If someone in your child’s family has had a Wilms’ tumor, then your child has a slightly increased risk of developing the disease. So family history plays a role in some cases
Wilms’ tumor occurs more frequently when certain abnormalities present at birth…
-Aniridia- rare eye condition the iris is partially or completely absent
-Hemi hypertrophy- abnormality occurs when one side of the body is noticeably larger than the other side
-Undescended testicles- one or both testicles fail to descend into the scrotum (cryptorchidism)
-Hypospadias- the urinary (urethral) opening is not at the tip of the penis, but is on the underside
THESE ARE NOT RISK FACTORS
Wilms’ can occur as part of rare syndromes, including…
- WARG syndrome: this syndrome includes Wilms’ tumor (W), Aniridia (A), abnormalities of the genitals (G) and urinary system, and mental retardation (R)
- Denys-Drash syndrome: includes Wilms’, kidney disease and male pseudo-hermaphroditism, undescended testicles and progressive renal failure
- Beckwith-Wiedemann syndrome: symptoms of this syndrome are omphalocele, large tongue (macroglossia), and enlarged internal organs
Assessment of Wilms’ Tumor
- Swelling or mass within the abdomen (mass is characteristically firm, contender, confined to one side, and deep within the flank)
- Abdominal pain
- Urinary retention or hematuria or both
- Anemia (caused by hemorrhage within the tumor)
- Pallor, anorexia, and lethargy
- Hypertension (caused by secretion of excess amounts of renin by the tumor)
- Weight loss and fever
- Symptoms of lung involvement, such as dyspnea, SOB, and pain in chest, if metastasis has occurred
Diagnosis and Testing for Wilms’ Tumor
- Physical Exam: Complete H&P is the best diagnostic tool for evaluation
- Blood/Urine test: blood test can’t detect Wilms’ but they can provide overall assessment of health. Urine tests can rule out a different type of cancer
- Imaging tests: Ultrasound is commonly the first test used, but a CT or MRI scan may also be done for confirmation
- Chest X-Ray, chest CT scan, chest MRI, bone scan to determine if it has spread beyond the kidneys
Staging Wilms’
The Treatment depends on the stage of the cancer, the type of cancer cell, and the child’s age and general health
- Stage 1: The cancer is found only in the kidney, and generally can be completely removed with surgery
- Stage 2: The cancer has spread to the tissues and structures near the kidney, such as at or blood vessels, but it can still be completely removed by surgery
Staging of Wilms’ cont…
- Stage 3: The cancer has spread beyond the kidney area to nearby lymph nodes or other structures within the abdomen, and it may not be completely removed by surgery
- Stage 4: The cancer has spread to distant structures, such as the lungs, liver or brain
- Stage 5: Cancer cells affect both kidneys. Rare
Treatment for Wilms’
- After staging and histology of the cell tissue is determined, the type treatment plan is formulated, usually by a pediatric oncologist. Standard treatment for Wilms’ tumor is surgery and chemotherapy. Radiation is used only in cases where the histology of the cells and staging requires
- Treatment is determined by staging and cell histology. Cell histology of the tumor reveals either favorable or unfavorable cells (both types are malignant). The long term prognosis is not as good in children who have anapestic, or unfavorable cells-but the prognosis in many is still good.
Therapeutic Management for Wilms’
Includes a combined treatment of surgery (partial to total nephrectomy) and chemotherapy with or without radiation, depending on the clinical stage and the histological pattern of the tumor
General treatment guidelines for Wilms’
- Stage one: Surgery (nephrectomy) 18 weeks or more chemo. 5 year survival > 95%. > 85% anapestic cells. About 5 clients out of 10 are treated in Stage 1.
- Stage 2: Surgery and abdominal radiation, 18-24 weeks chemo. 5 year survival > 95%. Anaplastic > 70%. 2 of 10 clients treated in this stage
Treatment guidelines cont..
- Stage 3: Surgery and abdominal radiation. 24 weeks of chemo. Sometimes radiation to shrink tumor then surgery. 5 year survival > 95%. Anaplastic > 60%. 2 if 10 treated in this stage
- Stage 4: Surgery and abdominal radiation. 24 weeks of chemotherapy and possible direct radiation of metastatic site. 5 year survival > 90%. Anaplastic < 20%. 1 of 10 at treatment.
- Stage 5: Highly individualized. If at all possible partial nephrectomy in both kidneys. The 5 year survival: Anaplastic < 20%, Non-anaplastic 80-90%
Types of Surgery in Wilm’s
Simple nephrectomy
Partial nephrectomy
Radical nephrectomy
Simple Nephrectomy
In this surgery, the surgeon removes the entire kidney. The remaining kidney can increase its capacity and take over the entire job of filtering the blood
Partial Nephrectomy
This involves removal of the tumor and part of the kidney tissue surrounding it. It’s usually performed when the other kidney is damaged (and stage 5) or has already been removed
Radical Nephrectomy
In this type of surgery, doctors remove the kidney and surrounding tissues, including the ureters and adrenal gland. Neighboring lymph nodes also may be removed
Chemotherapy
- Several chemo therapeutic agents are used to treat Wilms’ depending on child’s age, overall health status and stage and histology of tumor.
- General and common side effects are: nausea, vomiting, loss of appetite, hair loss, and low white blood cell counts
- Most side effects will improve after the drug is stopped, and may lessen during therapy
Common chemo agents to treat Wilms’
- Actinomycin D and Vincristine are usually effective.
- Other agents usually in late stages and/or anapestic cases:
- -Doxorubincin (cardiotoxicity), Etoposide (VP-16), Bone Marrow Suppression, and Cyclophosphamide (serious bladder side effects).
- A new drug, still in trails, Topotecan, is showing promise as treatment for anaplastic Wilms’ tumor.
