| Do intracranial neoplasms differ in Ollier disease and maffucci syndrome? An in-depth analysis of the literature. | |
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MedLine Citation:
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PMID: 19934970 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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OBJECTIVE: Ollier disease (OD) and Maffucci syndrome are closely related, very rare syndromes, that are both associated with multiple sites of chondrodysplasia. They differ primarily with the additional association of Maffucci syndrome with vascular abnormalities, including hemangiomas. Both are associated with chondrosarcomas and other nonsarcomatous neoplasms (NSN), but Maffucci syndrome is thought to have greater malignancy potential. We examined whether OD and Maffucci syndrome differ in the number, histology, in the location of intracranial malignancies, and in the demographics of such patients, as reported in the medical literature. METHODS: Relevant cases were identified by electronic searches on PubMed, SciSearch, Scientific Commons, Springer Link, and Google. Translate DotNet and Babelfish were used to translate non-English text. Unpaired Student's t tests were used to compare OD and Maffucci syndrome, and chondrosarcoma and NSN patients for mean age; Pearson chi analysis was used for comparisons of gender distribution, geographical distribution (by continent), site of lesion, and for OD versus Maffucci syndrome, tumor type (chondrosarcoma versus NSN). RESULTS: Forty-six patients with 47 intracranial malignancies were identified: 24 with OD, including 6 with a chondrosarcoma and 18 with an NSN; and 22 with Maffucci syndrome, including 13 with a chondrosarcoma, 8 with an NSN, and 1 with both. The 2 syndromes were statistically different in the distribution of chondrosarcomas versus NSNs (P = 0.002). All chondrosarcomas originated at the base of the cranium. All 18 NSNs in OD were of glial cell origin, but only 5 of 9 NSNs in Maffucci syndrome were glial; other tumors included pituitary adenoma, olfactory neuroblastoma, malignant chordoma, and spindle cell hemangioendothelioma. Patients with OD were more than 10 years younger than their Maffucci syndrome counterparts (24.7 versus 34.9 years; P = 0.002), as were patients with OD and chondrosarcoma versus those with Maffucci syndrome and chondrosarcoma (24.7 versus 36.2 years; P = 0.035). The 2 syndromes did not differ in overall sex distribution. OD and Maffucci syndrome differed in geographical distribution, with 10 Maffucci syndrome but no OD patients with malignancy either in Asia (7 patients) or South America (3 patients). Among NSNs, OD and Maffucci syndrome did not differ as to site of lesion within the brain. CONCLUSION: OD and Maffucci syndrome differ with respect to the distribution of intracranial malignancies by histology, and geographical and age distribution of cases, with OD patients younger by approximately a decade, and Maffucci syndrome patients more likely to live in Asia or South America. |
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Authors:
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Adrianna Ranger; Artur Szymczak |
Publication Detail:
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Type: Journal Article; Review |
Journal Detail:
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Title: Neurosurgery Volume: 65 ISSN: 1524-4040 ISO Abbreviation: Neurosurgery Publication Date: 2009 Dec |
Date Detail:
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Created Date: 2009-11-25 Completed Date: 2010-02-22 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 7802914 Medline TA: Neurosurgery Country: United States |
Other Details:
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Languages: eng Pagination: 1106-13; discussion 1113-5 Citation Subset: IM |
Affiliation:
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Department of Neurosurgery, Children's Hospital, London Health Sciences Center, London, Canada. aranger@uwo.ca |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Adult Brain Neoplasms* / complications, diagnosis Child Chondrosarcoma* / complications, diagnosis Databases, Factual / statistics & numerical data Enchondromatosis / complications*, pathology*, physiopathology Female Humans Male Young Adult |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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