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Virtual Pediatric Hospital: Paediapaedia: Osteochondromatosis Paediapaedia: Musculoskeletal Diseases

Osteochondromatosis

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed


Clinical Presentation:
Exostoses can present at any time from birth to the cessation of growth.

Etiology/Pathophysiology:
Due to a beaked failure of constriction with cortical overgrowth adjacent to the growth plate with subsequent eccentric bony growth from this beak in a direction away from the joint forming a tumor like excrescence that continues to grow until the growth plate closes. It is the most common skeletal tumor in childhood. Malignant degeneration occurs in 2-10% of cases, and should be suspected if there is rapid increase in size of an exostosis, especially in an adult.

Pathology:
Not applicable

Imaging Findings:
Most frequently seen in long tubular bones such as the distal femur, proximal tibia, proximal ulna, proximal femur, and distal forearm. Appears as a cystic excrescence projecting away sfrom the metaphysis that has its axis pointing away from the joint.

DDX:

References:

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