The association between intracranial tumours and multiple dyschondroplasia (Ollier's disease or Maffucci's syndrome): do children and adults differ?

In Ollier's disease (OD) and Maffucci's syndrome (MS), there is deforming dysplasia of cartilage, primarily but not exclusively involving the metaphyses and diaphyses of long bones. In a minority of patients with either of these rare syndromes, dysplasia can lead to sarcomatous degeneration, producing chondrosarcomas. There also appears to be an association with other neoplasms, which can include intracranial tumours. The primary objective of the current paper was to compare children/adolescents who have either OD or MS and an intracranial malignancy with their adult counterparts. All relevant cases in the medical literature were identified by electronically searching PubMed, SciSearch, Scientific Commons, Springer Link, and Google. Translate DotNet and Babelfish were used to translate non-English text. Non-parametric Pearson chi-square analyses were used to compare youths versus adults with respect to gender and geographic distribution (by continent), tumour histology and site of lesion, and the underlying enchondromatosis syndrome (OD vs. MS). All tests were 2-tailed, and P < 0.05 represented a statistically-significant difference. Forty-six patients with 47 intra-cranial malignancies were identified, with nine of the patients being 18 years old or less and categorized as youths. The incidence of intracranial chondrosarcomas peaked in the fourth decade of life, in parallel with the peak number of MS cases; conversely, both non-sarcomas and OD peaked in the third decade of life. Six of nine youths (67%) versus 17 of 36 adults with gender data (47%) were female (P = 0.30). There was no difference in geographic distribution by continent (P = 0.82). Four youths (44%) versus 16 adults (43%) had a chondrosarcoma (P = 0.95), and there was no statistically significant difference by tumour site (P = 0.42). However, seven (77%) of the youths had Ollier's disease as their underlying enchondromatosis syndrome, versus just 17 (46%) of the adults, a difference that approached statistical significance (P = 0.086). The association between enchondromatosis and intracranial malignancy seems to be roughly the same in youths versus adults, though Ollier's disease cases appear to predominate among youths.