Wilm's tumor is a condition unknown to most people, and for good reason: it is an incredibly rare form of cancer which generally just impacts children. Even so, it still needs to be taken seriously and the better it is understood by science and civilians alike, the better it can be detected and stopped.
Wilm's Tumor
Description
Wilm's tumor refers to a very rare type of kidney cancer that is also known as nephroblastoma, a type of cancer which can impact both kidneys, though usually first developing in just one. Because Wilm's tumor so frequently impacts children, doctors tend to think that "the tumor begins to grow as a fetus develops in the womb, with some cells that are destined to form into the kidneys malfunctioning and forming a tumor" (kidshealth.org, 2013). At the same time, even though this condition is more common to children, it can still occur in adults. It generally manifests between the ages of 3 and 4 and becomes less likely to occur around the age of five.
Symptoms and Signs
It's important to acknowledge that while this condition does occur with a set group of symptoms, some children experience no symptoms whatsoever. Another important aspect to remember is that children with this condition will appear outwardly very healthy, aside from the symptoms which are often very specific to this disease. According to the MayoClinic, some of the more common symptoms include: abdominal swelling, the presence of an abdominal mass one can feel, fever, blood in the urine (2011). The presence of any of these symptoms in a consistent or worrisome manner is enough reason to schedule an appointment with a doctor. Since this really is a rare condition, "the signs and symptoms associated with Wilm's tumor aren't specific to the condition and are much more likely to be caused by something else" (MayoClinic, 2011).
Patient Screening
Patient screening should definitely occur specifically with children who have a predisposition for developing this condition or who pose a high risk towards developing it. These generally include "most children with Beckwith-Wiedemann syndrome, WAGR syndrome, Denys-Drash syndrome, idiopathic hemihypertrophy, or sporadic aniridia) should be screened with ultrasound every 3 months at least until they reach age 8 years" (cancer.gov, 2013). For instance, children who have a hemihypertrophy also are at an aggravated risk for getting tumors of the liver or adrenal arena; thus ultrasounds are crucial for proper screening: an abdominal ultrasound is suggested, with a renal ultrasound after the age of four (cancer.gov, 2013).
Etiology/Pathophysiology
Fundamentally, Wilm's tumor is seen as developing as a result of an alteration of genes which are generally accountable for genitourinary development (medscape, 2013). "Examples of common congenital anomalies associated with Wilm's tumor are cryptorchidism, a double collecting system, horseshoe kidney, and hypospadias. Environmental exposures, although considered, seem relatively unlikely to play a role" (medscape, 2013). The WT1 gene and other genetic loci have also been targeted as playing a strong role for the development of the condition; the other genetic loci responsible are seen as playing a role in this condition because of the fact that certain families seem more susceptible to it than others (medscape, 2013).
Diagnosis
Diagnosis is made as a result of a range tests: an overall physical exam will need to be conducted, as will blood and urine tests, and imaging tests: "Imaging tests that create pictures of your child's kidneys can help your doctor determine whether your child has a kidney tumor. Imaging tests may include ultrasound, computerized tomography (CT) and magnetic resonance imaging (MRI)" (MayoClinic, 2011). During this process, surgery might be suggested as a result of the doctor needing to determine if the entire tumor is cancerous -- thus, the tumor would be removed during surgery and then analyzed in a laboratory (MayoClinic, 2011).
Treatment
Once the child has been fully diagnosed with the cancer, the doctor will properly determine the staging: he or she might need the assistance of a chest X-ray or CT scan to help elucidate the staging process. Furthermore, the patient will generally be exposed to the general types of standard cancer treatment -- surgery and chemotherapy. "The stage of the tumor and appearance of the cancer cells under a microscope help determine whether your child also needs radiation therapy. At this point, your doctor may tell you the tumor appears to be either favorable or unfavorable (anaplastic) -- the histology of the tissue. Children whose tumors have a favorable histology have better survival rates. However, many children with unfavorable histology also have good outcomes" (MayoClinic, 2011). Surgeries like a simple nephrectomy, partial nephrectomy or a radical nehprectomy can help remove the cancerous cells by removing parts of the kidney (MayoClinic, 2011). In the case that both kidneys need to be removed, dialysis will have to be received until the child can receive a kidney transplant.
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