Wilms tumor: epidemiology, pathophysiology, diagnosis, and treatment

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).

Prognosis

The prognosis is generally very connected to the staging system of the disease that the doctor is in charge of for the viability of the cure. The staging system helps in elucidating the prognosis; for instance, a child with a truly aggressive condition should receive an intensive regimen of medication so that a cure is best achieved (kidshealth.org). On the other hand, a child with less-invasive diseases should get the least amount to minimize the long-term side effects from the toxicity (kidshealth.org).

Prevention

Unfortunately, there's no foreseeable means of preventing Wilm's tumor: if a child has signs or symptoms which indicate its presence or put the child at an exacerbated risk for the condition then periodic ultrasounds of the kidneys will be necessary to chart abnormalities and other factors (MayoClinic, 2011). While this is not a preventative tool it can contribute to early detection which can be incredibly successful in stopping the disease or fighting it more effectively.