Wilms tumor - with hemorrhage

Case contributed by Dr Joe Mullineux

Presentation

Previously fit and well young girl presents acutely unwell with diffuse abdominal pain, pallor, tachycardia and labile BP.

Patient Data

Age: 5 years
Gender: Female
Ultrasound

Selected images from an emergency ultrasound performed by the on call radiologist in a general hospital (without pediatric surgical services) reveals a large heterogenous mass occupying the majority of the right upper quadrant.

Within the soft tissue mass centrally there is echogenic layered material thought to be hemorrhage. The mass is difficult to locate to one organ due to its size however the mass is positioned above the right kidney wand unable to separate from the right kidney.

The differential diagnosis included renal tumor, large adrenal hemorrhage or mass and late presenting neuroblastoma.

An urgent CT with arterial phase was advised to assess for active hemorrhage and diagnosis due to the patient's condition, whilst a transfer to a specialist pediatric center was arranged by the referring emergency department.

CT

Non- contrast phase CT shows the large mass in the RUQ with layered hyperdensity central in keeping with internal hemorrhage.

Biphasic contrast injection or "split bolus" injection was used to assess both portal venous and arterial phase in a single acquisition to reduce radiation dose.

The post contrast images show no active contrast extravasation to suggest active bleeding. The soft tissue vascular mass arises from the right kidney as evidenced by the "claw sign". There is no intra-lesional fat or calcification.

The mass also appears in close contact with the liver were focal areas appeared to be crossing the liver capsule in the right lobe which is worrying for local invasion of the liver.

Of note IVC is significantly compressed by the mass which may have been contributing to cardiovascular instability due to lack of preload.

No metastatic disease demonstrated.

Case Discussion

Once the lesion was proven to arise from the kidney it was almost definitely a Wilm's tumor which is the most common renal tumor in this age group and was confirmed on surgical resection.

I suspect the spontaneous internal hemorrhage into the tumor precipitated a rapid increase in tumor volume resulting in the acute presentation. After the diagnostic CT, the patient was taken by ambulance to the nearest pediatric specialist center for full work up and definitive management.

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Case information

rID: 60517
Published: 9th Jun 2018
Last edited: 14th Aug 2019
Inclusion in quiz mode: Included

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