Mazabraud syndrome

Case contributed by Dr George Nita

Presentation

Painless, slowly enlarging mass on right arm causing occasional paraesthesia.

Patient Data

Age: 46
Gender: Male
X-ray

Anteroposterior radiograph of the right arm shows areas of minor expansile diaphyseal lesions with well marinated borders and ground glass matrix with some sclerosis in the proximal two-thirds of the humerus. Changes are suggestive of underlying fibrous dysplasia.

MRI

MRI shows a well-defined solitary soft tissue mass, lying within the lateral aspect of the triceps muscle, not related to the neurovascular bundle. Significant marrow signal abnormality is demonstrated throughout the humerus extending from the humeral head to the distal humerus.

Case Discussion

This 46-year-old male was referred by his general practitioner for the appearance of a slowly enlarging mass on his right arm that had been present for 6 months with occasional paraesthesia in the radial nerve distribution. He had no history of trauma and was otherwise asymptomatic. The patient presented 10 years ago for a lump in the medial aspect of his right upper arm which was subsequently excised and the diagnosis of Mazabraud’s syndrome was established following biopsy.

The imaging findings are similar to previously reported cases in the literature. MRI shows significant marrow signal abnormality throughout the humerus. In addition, a well-defined soft tissue mass, lying within the lateral aspect of the triceps muscle at the level of the midshaft of humerus appears of intermediate signal intensity on T1-weighted images, high signal intensity on the fat sat proton density images and exhibits some internal enhancement post-gadolinium 

A Tru-cut needle biopsy was carried out and the histopathologic examination confirmed cores of skeletal muscle interfacing with an ill-defined myxocollagenous lesion displaying the features of an intramuscular myxoma.

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Case information

rID: 44011
Case created: 2nd Apr 2016
Last edited: 3rd Apr 2016
Inclusion in quiz mode: Included

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