Chondrosarcomas of the base of the skull in Ollier's disease or Maffucci's syndrome Three Case Reports and Review of the Literature

Maffucci' s syndrome complicated by intracranial chondrosarcoma: two new illustrative cases

“…In 1900, Ollier 2 described a similar congenital enchondromatosis without hemangiomas. In reviewing the literature on Maffucci's syndrome, the authors highlight the difficulty involved in making a definitive diagnosis in rare conditions with similar pathology 3 .To the best of our knowledge, these two conditions as reported in the literature were thought to be distinct entities, but some authors suggest that both syndromes represent a spectrum of the same disease process rather than two distinct diseases 4,5 . Actually, it has been suggested that all patients diagnosed to have Ollier's disease should be screened with total body magnetic resonance imaging (MRI) to exclude the presence of hemangiomas and other associated occult malignancies 3 .…”

“…Schwartz et al 17 using the life- 13 when they studied 105 cases and found the incidence of sarcomatous transformation of the enchondromas to be 15 per cent, and the overall incidence of malignant tumors in this group of 105 patients to be as high as 23 per cent. Some authors have reported an incidence of associated malignancies of 50% or more 4,6 . Among cases of malignancies associated with Maffucci's syndrome, less than 10% were located in the base of the skull and less than half of these tumors were chondrosarcomas 4 .…”

“…Some authors have reported an incidence of associated malignancies of 50% or more 4,6 . Among cases of malignancies associated with Maffucci's syndrome, less than 10% were located in the base of the skull and less than half of these tumors were chondrosarcomas 4 . This incidence appears to be lower in Ollier's disease.…”

“…Mellon et al 14 In several case reports, we found that at time of the diagnosis of chondrosarcoma, the patient was first considered to present Ollier's disease, but the concomitant diagnosis of a hemangioma indicated a correct diagnosis of Maffucci's syndrome [3][4][5][6]14,[26][27][28] .…”

“…Others believe it is a congenital mesodermal dysplasia 3,10 . In a review of the literature, we were able to find 209 reports of Maffucci's syndrome 4,5,[11][12][13] . Twenty-sev-en of the reported patients had intracranial lesion, but only 12 presented with intracranial chondrosarcoma.…”