What's the Diagnosis - Case 37

History 52 year old man with increasing right shoulder pain.

What’s the Diagnosis - Case 37

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Findings Multiple osseous lesions are seen but noted only on the right side of the body. The lesions are lobulated, heterogenous, and with high signal intensity on MR imaging .

Punctate calcification is seen of multiple masses on the radiographs and CT imaging. Over time there is expansion of the osseous masses with subsequent resection and orthopedic instrumentation.

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Diagnosis: Ollier’s Disease with malignant transformation A non-hereditary condition of multiple enchondroma or enchondromatosis yielding expansion and deformity of the bone and often rendering short bones or limb length discrepancies. At times the lesions are pedunculated, simulating osteochondroma and referred to as enchondroma protuberans. Lesions tend to predominate in the long bones as well as within the metacarpals/metatarsals. Lesions in the flat bones are not as common. In childhood, lesions are subject to fracture and in adulthood there is an increased risk of malignant transformation. After a child has reached skeletal maturity, the lesions should no longer grow. Continued growth with destruction of the underlying bone indicates malignant transformation which is said to occur in up to 30% of individuals. Typically, malignant transformation is to a chondrosarcoma. Lesions are treated with excision and patients are closely monitored for potential malignant transformation as in this case.

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Resources

• Resnick. Diagnosis of Bone and Joint Disorders. 4th Ed. 2002

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