Here we present an interesting case of Maffucci syndrome with a history of malignant transformation of enchondromas
and recurrent hemangiomas that required multiple prophylactic surgical excisions.
2) Although they usually occur spontaneously, they may also arise as a result of malignant degeneration of an endochondroma, as seen in both Maffucci syndrome (a mesodermal dysplasia that causes enchondromas
and hemangiomas) and Ollier disease (which manifests as multiple enchondromas
Ollier's disease also confers an increased risk of malignancy, with transformation of the enchondromas
into chondrosarcomas being noted, most often around age 40.
In fact, radiographs of the hands and feet are often pathognomonic in Maffucci syndrome, showing multiple soft tissue masses containing phleboliths in addition to the multiple enchondromas
are usually solitary benign lesions, consisting of ectopic hyaline cartilage deposits in intramedullary bone.
Some lesions, such as solitary bone cysts (Figure 4), enchondromas
(Figure 5), EG and Ewing's sarcoma, tend to be centrally located.
are hyaline cartilage tumors in the medullary canal.
of the skull base are exceedingly rare.
Enchondromatosis, or Ollier's disease, is a rare, nonhereditary dyschondroplasia characterized by multiple, asymmetrical enchondromas
throughout the skeleton.
If arising within the medullary cavity, they are known as enchondromas
Chondrosarcomas may arise de novo (primary), or superimposed (secondary) upon preexisting cartilaginous lesions, such as enchondromas
Cartilaginous tumors (such as osteochondromas, enchondromas
, and chondrosarcomas) may produce irregularly shaped calcifications within their matrix that resemble popcorn on imaging studies (Figure 16).