RSNA Case Collection: Ollier Disease Chondrosarcomatous Degeneration.

Clinical history and presentation:

This is a patient who was born in Scotland without significant family medical history. Early in childhood, he noticed small bony bumps over his right wrist. Upon medical consultation, wrist radiographs were performed and were diagnostic of enchondroma. 

Over time, he developed other bony deformities of his extremities, ribs, and maxillary sinuses. These lesions were monitored via radiography and some required reconstructive surgery to help with functional outcome. 

Once the patient transitioned his care to our institution, he described having new pain associated with his right hand/wrist deformities. This is the region of interest explored in this case.

Pathology: Right hand

The specimen contains multiple separate nodules of conventional type chondrosarcoma. These span up to 13 cm in greatest dimension and involve the distal radius, metacarpals of the thumb/index/long fingers, and phalanges of the thumb/index finger.

Physical exam: The distribution of disease is more prominent on the right side of the body. Status post amputations of the right length, ring, and little fingers. His thumb, palm, and wrist have multiple firm masses that cause skin tension and are tender to palpation.

Figure legend: 

There are multiple lucent lesions, some with the classic rings and arcs calcification suggestive of chondroid lesions. There are areas of cortical destruction suggestive of extra-osseous extension of the lesions, which can be seen in the setting of malignant degeneration into chondrosarcoma.