Haemophilic pseudotumours are rare complications of haemophilia consisting of a progressive cystic swelling of muscle and/or bone due to repeated bleeding, occurring in <2% of haemophiliacs.
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Epidemiology
Haemophilic pseudotumours are reported in 1-2% of patients with haemophilia.
Clinical presentation
The clinical presentation varies considerably depending on location of pseudotumour, but is generally slowly progressive and chronic.
Pathology
Most develop in the muscles of the pelvis and lower extremity, where the large muscles have an abundant blood supply, or in bone following intraosseous bleeding.
Radiographic features
Ultrasonography, CT and MRI have significant roles in detecting pseudotumours, especially when they are in the pelvis.
Plain radiograph
X-ray is useful in diagnosing intraosseous pseudotumours. They appear as well-defined, unilocular or multiloculated, lytic, expansile lesions of variable size. It can involve metaphysis, diaphysis and epiphysis of long bones.
Other findings include endosteal scalloping, perilesional sclerosis, cortical thinning or thickening, trabeculations and septations. Pathological fractures can also be present.
Ultrasound
In the acute and subacute stages, sonography shows a central anechoic area with increased echoes behind the lesion, caused by fluid in the pseudotumour. A distinct plane separates the mass from the surrounding muscles.
CT shows the thick wall more consistently than does ultrasonography. Multiple irregular echoes represent solid material that cannot be documented on CT. Differential diagnosis from abscess may be difficult.
CT
CT is useful for detecting both the extent of soft-tissue masses and the involvement of bone. Pseudotumours contain coagulated blood and are surrounded by a thick wall.
Contrast-enhanced CT is useful for determining the thickness of the wall. In the acute stage, the centre of the pseudotumour is hypodense on CT, but the periphery is isodense and indistinguishable from surrounding muscle.
MRI
The hypointense rim of a pseudotumour on both T1 and T2-weighted images consists of fibrous tissue that contains haemosiderin.
Treatment and prognosis
The ideal treatment is prevention with adequate management of acute bleeds (e.g. rest, factor replacement, physiotherapy, etc.) 6. If development of a pseudotumour occurs, multiple management options have been described, including low-dose radiotherapy, percutaneous curettage, surgical resection, or filling with bone graft or other compounds 5,6.
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