Wilms’ Tumor

Wilms’ tumor is the 5th most common pediatric malignancy and the most common renal tumor in children. Wilms’ tumor occurs most often in children between the ages of 2 and 5 years and rarely in adults. Approximately 500 new cases are diagnosed in the U.S. annually. The majority of children present with an abdominal mass. Other symptoms may include hypertension, fever from tumor necrosis, hematuria and anemia. The majority (75%) occur in otherwise healthy children. A minority (25%) are either familial or associated with other developmental abnormalities such as hemihypertrophy, Beckwith-Wiedemann syndrome, sporadic aniridia, neurofibromatosis, and cerebral giantism. Wilms tumors are highly responsive to treatment, with about 90% of patients surviving at least five years.

Image from Toshiba America Medical Systems (link)

Wilms’tumors are usually very large which distorts and displaces the collecting system and capsule. In 40% of patients the tumor may be seen extending into the renal vein and is bilateral in 6% of cases. Local tumor invasion is rare but the tumor cells may spread to the lungs, liver or nearby lymph nodes. Sonographically, the tumor is hyperechoic and homogeneous. Hypoechoic areas can be present due to hemorrhage and necrosis. Calcifications are seen infrequently, in less than 15%.

Treatment for Wilms tumors includes radical nephrectomy, chemotherapy, and abdominal radiation for higher stage disease.

Prognosis for Stage II Wilms’ tumor is excellent — 96% 4 year survival.

Wilms’ tumor may recur after several years, therefore follow up imaging surveillance is continued for at least 5 years.  (reblogged from Clinical Case Studies Toshiba America Medical Systems)

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