Enchondroma of the petrous bone and parasellar area in Maffucci's syndrome.

Maffucci's syndrome is a congenital disorder characterized by multiple hemangiomas and enchondromatosis that can be present at birth or can appear during infancy or later in childhood. The cavernous or capillary hemangiomas of the skin in these patients are usually unilateral. Hemangiomas can also occur in some viscera and mucous membranes, often in the mouth, esophagus, and ileum. The skin lesions do not necessarily overlie those of bone. [1]

Enchondromas sometimes cause dyschondroplasia of the long bones during childhood. They can also appear in the small bones and rarely at the base of the skull, as in the case presented here (figure 1). At least one-fourth of affected patients develop malignancies, especially chondrosarcomas. Other connective tissue neoplasms can arise, such as gliomas (figure 2), fibrosarcomas, angiosarcomas, lymphangiosarcomas, and others. [2] This syndrome is inherited as an autosomal-dominant trait.

From the Department of Radiology, Louisiana State University Health Science Center. New Orleans (Dr. Palacios and Dr. D'Antonio), and the Department of Radiology and Otolaryngology, University of Illinois Medical Center, Chicago (Dr. Valvassori).

References

(1.) Sun TC, Swee RG, Shives TC, Unni KK. Chondrosarcoma in Maffucci's syndrome. J Bone Joint Surg Am 1985;67:1214-9.

(2.) Chakrabortty S, Tamaki N, Kondoh T, et al. Maffucci's syndrome associated with intracranial enchondroma and aneurysm: Case report. Surg Neurol 1991;36;216-20.