IS Case 330: Multiple osteocartilaginous exostoses

Amira Farouga, MD

Imaging Sciences URMC 2009
Publication Date: 2009-09-15

History

Patient is an 23-year-old female with multiple fractures from motor vehicle collision (MVC).

Findings

Multiple osteochondromas are seen around the left knee joint, some are sessile others are pedunculated and directed away from from the knee joint.

Diagnosis

Multiple osteocartilaginous exostoses

Discussion

Multiple osteocartilaginous exostoses, also known as multiple hereditary osteochondromata, familial osteochondromatosis or diaphyseal aclasis, is a hereditary autosomal-dominant disorder. There is a decided 2:1 male predilection. The knees, ankles, and shoulders are the sites most frequently affected by the development of multiple osteocartilaginous exostoses.

The radiographic presentation is characteristic according to whether the lesions are sessile, more frequent, with broad base attached to the cortex or pedunculated, with slender pedicle usually directed away from the neighboring growth plate.

There are a greater incidence of growth disturbance in multiple osteocartilaginous exostoses than in solitary osteochondroma. Growth abnormalities are seen in the forearms and legs.

Malignant transformation to chondrosarcoma is also more common.

References

  1. Epstein DA, Levin EJ. Bone scintigraphy in hereditary multiple exostoses. AJR Am J Roentgenol. 1978 Feb;130(2):331-3. PMID: 414588

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