Medical Central Resource

Wilm's tumor Stage V

Kristen Fults-Ganey

3/25/97

Presentation

7 year old female with abdominal mass.

Imaging Technique

Plain X-ray CT Ultrasound

Imaging Findings

KUB: There is a medial displacement of the stomach and inferior displacement of the bowel in the left upper quadrant suggestive of a mass in the expected region of the left kidney.
Abdominal CT: There is a right hypodense renal mass in the posterolateral aspect of the mid kidney with vague areas of increased density which may represent calcification. There is a large heterogenous left renal mass with no areas of calcification.
Renal US: Image of the left kidney demonstrates a large multicystic mass. Transverse image of the right kidney demonstrates an echogenic mass in the mid pole.

Diagnosis

Wilm's tumor Stage V

Discussion

Wilm's tumor is the most common malignant abdominal neoplasm in children 1-8 years of age and the third most common malignancy in childhood. Fifty percent are diagnosed between 1-3 years of age and 75 % before 5 years of age. It is uncommon in the first year of life and rare beyond young adulthood. Common clinical manifestations include palpable abdominal mass, hematuria, and systemic hypertension.
The tumor is derived from primitive nephrogenic blastema and the neoplasm is surrounded by a fibrous pseudocapsule. Hemorrhage and necrosis with cavitation of the mass is common and calcification is seen in 5% on plain film and in 15% on CT.. The tumor is bilateral in 5-10% of the cases. Local venous spread and distant metastases, especially to the lung and liver, are common.
There is an increased incidence in Wilm's tumor in patient's with sporadic aniridia, trisomies 8 and 18, Turner's syndrome, hemihypertrophy, Beckwith-Wiedemann syndrome, Drash syndrome, and nephroblastomatosis.
The prognosis depends on tumor histology, staging, and age of patient at time of diagnosis. The prognosis is more favorable in patients less than two years of age. Histologic patterns include well differentiated, favorable histology and poorly differentiated, unfavorable histologies including anaplastic, clear cell sarcomatous, and rhabdoid cell types.

Submitted by: Kristen Fults-Ganey,Capt,USAF,MC,Wilford Hall Medical Center
Reviewed by: Cindy Taylor,LtCol,USAF,MC,Wilford Hall Medical Center

References