Wilms tumour - with haemorrhage

Case contributed by Joe Mullineux
Diagnosis almost certain

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 paediatric 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 haemorrhage. 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 tumour, large adrenal haemorrhage or mass and late presenting neuroblastoma.

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

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

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 tumour which is the most common renal tumour in this age group and was confirmed on surgical resection.

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

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