Wilm’s Tumor

Background:

 Wilm’s Tumor or nephroblastoma is cancer of the kidneys that typically occurs

in children, rarely in adults. Its common name is an eponym, referring to Dr. Max Wilms, the
German surgeon (1867–1918) who first described this kind of tumor.

Approximately 500 cases are diagnosed in the U.S. annually. The majority (75%) occurs
in otherwise normal children; a minority (25%) is associated with other developmental
abnormalities. It is highly responsive to treatment, with about 90% of patients surviving at least
five years.

Etiology:

Wilms tumor may arise in 3 clinical settings, the study of which resulted in the discovery
of the genetic abnormalities that lead to the disease. Wilms tumors can arise sporadically, can
develop in association with genetic syndromes, or can be familial. Although some of the
molecular biology of Wilms tumor is coming to light, the exact cellular mechanisms involved in
the etiology of the tumor are still being investigated.

Signs and symptoms/ Clinical Manifestations:

...More common findings:

Palpable abdominal mass

Gross Hematuria (onset: 9 mos), Flank pain, Fever , Weight loss, Cachexia fatigue, HPN,
amyloidosis, thromblophlebitis, anemia,erythrocytosis, hypercalcemia, abnormal serum liver
profile, elevated ESR,

…Less frequent findings:

Peripheral neuropathy, inferior vena cava obstruction, priapism, variocele, hydronephrosis (if
tumor blocks ureteropelvic junction)

Labs and Diagnostics:

Plasma erythropoietin, renin, chorionic gonadotropin ang prostaglandin are elevated

IV Pyelogram
UTZ

CT Scan

Nephrotomography

Nursing Management:

PRE- OP: Inc fluid intake if indicated, emotional support

POST-OP:
V/S, WOF signs of hemorrhage, pneumothorax

NANDA problems:

Anxiety r/t threat of death

Risk for injury
Interrupted family process r/t expensive treatments

Knowledge deficit of the disorder and therapy

Prognosis:

Tumor-specific loss-of-heterozygosity (LOH) for chromosomes 1p and 16q identifies a subset of

Wilms' tumor patients who have a significantly increased risk of relapse and death. LOH for
these chromosomal regions can now be used as an independent prognostic factor together with
disease stage to target intensity of treatment to risk of treatment failure. Genome-wide copy
number and LOH status can be assessed with virtual karyotyping of tumor cells (fresh or
paraffin-embedded). The overall prognosis with surgical removal is positive. Early removal
tends to promote positive outcomes.