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Case Reports
. 2010 Jan;83(985):e10-3.
doi: 10.1259/bjr/14078580.

Odontogenic myxofibroma synchronous with primary angiosarcoma of the spleen

Affiliations
Case Reports

Odontogenic myxofibroma synchronous with primary angiosarcoma of the spleen

A Hadidy et al. Br J Radiol. 2010 Jan.

Abstract

Odontogenic myxofibroma of the temporomandibular joint (TMJ) is a rare tumour; moreover, primary splenic angiosarcoma (PAS) in paediatric patients is extremely rare. We report on a 15-year-old boy who presented with right TMJ swelling and subsequently developed epigastric and right upper quadrant pain. The TMJ swelling proved to be odontogenic myxofibroma and the abdominal pain was a result of primary splenic angiosarcoma with hepatic metastasis. We report for the first time the synchronous presentation of PAS and odontogenic myxofibroma in a paediatric patient, and we describe the radiological features along with the histological diagnosis and clinical outcome. Uptake in (18)F-2-fluoro-2-deoxy-D-glucose positron emission tomography is also described for the first time for both these tumours.

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Figures

Figure 1
Figure 1
Abdominal CT scan in the (a) portovenous and (b) delayed venous phases showing the hypodense splenic lesion (arrows) and multiple liver lesions.
Figure 2
Figure 2
Whole-body 18F-2-fluoro-2-deoxy-D-glucose (18FDG) positron emission tomography (PET) showing focally increased uptake in the right mandible (arrow) and increased uptake in both the liver and spleen.
Figure 3
Figure 3
MRI of the temporomandibular joints — (a) axial T1, (b) coronal T2 fat-saturated and (c) coronal T1 post-contrast scans — showing the right mandibular mass lesion (arrows).
Figure 4
Figure 4
Angiosarcoma of the spleen. The tumour has (a) sinusoidal and (b) cavernous growth patterns (haematoxylin and eosin stain, ×200)
Figure 5
Figure 5
Odontogenic myxofibroma. The high magnification shows a loose arrangement of spindle or stellate cells in a myxomatous background. Also of note is the prominent vascularity (haematoxylin and eosin stain, ×200).

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