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Review
. 2012 Aug;85(1016):1107-11.
doi: 10.1259/bjr/12938443. Epub 2012 Jan 17.

Imaging features of paediatric haemophilic pseudotumour of the maxillary bone: report of three cases and review of the literature

B T Yang  1 , Y Z Wang, X Y Wang, Z C Wang
Affiliations
Review

Imaging features of paediatric haemophilic pseudotumour of the maxillary bone: report of three cases and review of the literature

B T Yang et al. Br J Radiol. 2012 Aug.

Abstract

Objectives: Haemophilic pseudotumour (HP) is an extremely rare lesion. The purpose of this study was to describe the CT and MRI features of maxillary bone HPs and introduce the key points to differentiate HP from the mimicking entities in the region.

Methods: We retrospectively reviewed three paediatric patients with histology-proven HPs arising from the maxillary bone. All three patients underwent CT and/or MRI. Combined with six previously reported cases in the literature, the imaging features were comprehensively analysed.

Results: All HPs showed a well-demarcated, multilobulated expansile osteolytic lesion in the maxillary bone. On non-enhanced CT, HPs appeared of mixed density relative to grey matter. The lesions appeared to have markedly heterogeneous signal intensity on both T(1) and T(2) weighted images, with septa-like enhancement following the administration of contrast material, which corresponded to blood products in various stages of evolution. The lesions caused cortical thinning and even focal disappearance and multiple bone septa were identified within the involved maxillary bone. Some HPs were associated with radiated periosteal proliferation, which can easily be misdiagnosed as a malignant bone tumour.

Conclusion: A high index of suspicion for HP and a familiarity with imaging findings may help to accurately diagnose this rare entity.

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Figures

Figure 1
Figure 1
Case 1. (a) Coronal T1 weighted image shows mixed-signal soft tissue with multiple hyperintense signal areas. (b) Axial T2 weighted image demonstrates mainly hyperintense signal with hypointense signal rim of the lesion.
Figure 2
Figure 2
Case 2. (a) Coronal CT image shows a well-defined, multilobulated soft-tissue mass with diffuse cortical thinning with focal sclerosis. (b) Coronal T1 weighted image shows a mixed-signal lesion with hyperintense signal areas. (c) The lesion has mainly hyperintense signal with hypointense signal rim on the axial T2 weighted image. (d) Coronal contrast-enhanced T1 weighted image with fat saturation shows moderate septa-like contrast enhancement of the lesion. (e) Coronal T1 weighted image shows a recurrent lesion in the primary site. (f) Coronal T1 weighted image shows no evidence of recurrence.
Figure 3
Figure 3
Case 3. Axial CT image shows a well-defined, lobulated soft-tissue mass with fluid–fluid levels in the left maxillary bone.
See this image and copyright information in PMC

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