Adamantinoma

Case contributed by Dr Nicoletti Domenico Nicoletti

Presentation

Sudden pain with functional impotence of the left leg.

Patient Data

Age: 7 years
Gender: Male

Left leg

x_ray

Mildly displaced pathologic fracture of the middle third of the tibial shaft where an elongated multilocular lesion. There is endosseous scalloping and cortical remodeling. The transition zone is narrow. Separate lesions are also detectable in the lower middle third of the fibular shaft.

Left leg

x_ray

Note the rapid healing with extensive callus after 35 days.

Left leg

mri

Central well-defined expansile lesion with pathological fracture, hypointense on T1 weighted images with heterogeneously hyperintense signal on T2/STIR images. Fat suppressed post-contrast enhanced T1 shows avid, heterogeneous internal enhancement.

Fluoroscopy

Biopsy was performed.

pathology

Bone biopsy report.

Case Discussion

Adamantinomas are rare, low-grade malignant tumors of unknown etiology that usually involve the tibia, fibula, or both. Adamantinoma appears as a central or eccentric, multilocular ("soap bubble" appearance), slightly expansile, sharply or poorly marginated lytic lesion. There are satellite lesions in continuity with the major lesion. Often with reactive bone sclerosis and bowing deformity of the tibia. In the fibula the lesions are more centrally located with sharply demarcated lytic foci with sclerotic margins. The fibular lesions are typically with expansion of the bone contour without bowing deformity.

MRI demonstrates the intraosseous and extraosseous involvement. The differential diagnosis includes osteofibrous dysplasia (OFD), fibrous dysplasia, ABC, chondromyxoid fibroma and chondrosarcoma. A ground-glass appearance with intralesional calcifications suggests fibrous dysplasia whereas the presence of small satellite radiolucent foci is more characteristic of adamantinoma. Surveillance in OFD, with biopsy in case of onset of pain or increased tumor volume.

 

Case courtesy Dr. Paolo Cucchi Dr Simone Barbieri

Radiographer: TSRM Fabio Imola

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