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The Journal of Bone and Joint Surgery, Vol 69, Issue 2 269-274, Copyright © 1987 by Journal of Bone and Joint Surgery, Inc
The malignant potential of enchondromatosis
HS Schwartz, NB Zimmerman, MA Simon, RR Wroble, EA Millar and M Bonfiglio
In a tri-institutional, retrospective study with long-term follow-up,
forty-four patients who had multiple enchondromas were identified.
Thirty-seven patients did not have hemangiomas (Ollier disease) and seven
did (Maffucci syndrome). Of the thirty-seven patients who had Ollier
disease, a low-grade chondrosarcoma developed in four; an astrocytoma, in
one; and a granulosa-cell ovarian tumor, in one. In four of the seven
patients who had Maffucci syndrome, there were six low-grade
chondrosarcomas, one high-grade osteosarcoma, one pancreatic
adenocarcinoma, one biliary adenocarcinoma, and one astrocytoma. None of
the patients in either group died of the skeletal sarcoma, but four of five
patients who had a non-skeletal malignant lesion died. From life-table
analyses of these patients, we estimated that the incidence of secondary
chondrosarcoma in patients who have Ollier disease is about 25 per cent at
the age of forty years, and that malignant degeneration is almost a
certainty in patients who have Maffucci syndrome. We concluded that
periodic surveillance of the brain and abdomen for occult malignant lesions
is indicated in patients who have enchondromatosis.
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