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The association between intracranial tumours and multiple dyschondroplasia (Olliers disease or Maffuccis syndrome): do children and adults differ?
Journal of Neuro-Oncology, 06/16/09

Ranger A et al. - In a trial to compare children/adolescents who have either Ollier’s disease (OD) or Maffucci’s syndrome (MS) and an intracranial malignancy with their adult counterparts, it was concluded that the association between enchondromatosis and intracranial malignancy seems to be roughly the same in youths vs adults, though Ollier’s disease cases appear to predominate among youths.

• 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 vs 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.

• 46 pts with 47 intra-cranial malignancies were identified, with 9 of the pts 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.
• 6 of 9 youths (67%) vs 17 of 36 adults with gender data (47%) were female.
• There was no difference in geographic distribution by continent.
• 4 youths (44%) vs 16 adults (43%) had a chondrosarcoma, and there was no statistically significant difference by tumour site.
• 7 (77%) of the youths had Ollier’s disease as their underlying enchondromatosis syndrome vs just 17 (46%) of the adults, a difference that approached statistical significance.

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