DO INTRACRANIAL NEOPLASMS DIFFER IN OLLIER DISEASE AND MAFFUCCI SYNDROME? AN IN-DEPTH ANALYSIS OF THE LITERATURE
Notice bibliographique
Résumé
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.
Récupéré en direct depuis OpenAlex et désinversé. Les résumés ne sont pas conservés dans cette base de données : les index inversés représentent 8,6 Go des 9,3 Go de texte de la base, et le serveur dispose de 13 Go libres.
Comment cette classification a été obtenuedéplier
Prédiction distillée sur la base complète
Imitation des enseignantsNi prévalence calibrée, ni vérité terrain. Validation humaine à venir. Apprise à partir de 10 348 étiquettes directes de Codex et de 10 348 étiquettes directes de Gemma. Le mode candidate est l'union des têtes enseignantes seuillées; le consensus est leur intersection. Ces sorties portent le statut machine_predicted_unvalidated et ne sont ni des étiquettes humaines ni des étiquettes directes de modèles de pointe.
Scores Codex et Gemma par catégorie
| Catégorie | Codex | Gemma |
|---|---|---|
| Métarecherche | 0,000 | 0,000 |
| Méta-épidémiologie (sens strict) | 0,000 | 0,000 |
| Méta-épidémiologie (sens large) | 0,003 | 0,001 |
| Bibliométrie | 0,001 | 0,002 |
| Études des sciences et des technologies | 0,000 | 0,000 |
| Communication savante | 0,000 | 0,000 |
| Science ouverte | 0,000 | 0,000 |
| Intégrité de la recherche | 0,000 | 0,001 |
| Charge utile insuffisante (le modèle a refusé de juger) | 0,000 | 0,000 |
Scores machine (provisoires)
Les deux têtes enseignantes du modèle étudiant, lues sur ce travail. Un score ordonne la base pour la relecture; il n'affirme jamais une catégorie, et le statut de validation accompagne chaque rangée tel quel.
Scores de référence d'un modèle non mature (critères de maturité non atteints, 7 itérations). Un score ordonne; il n'affirme jamais une catégorie.
score_only:v0-immature-baseline · tel quel depuis la passe de notation : score_only signifie que le nombre peut ordonner les travaux, et qu'aucune étiquette de catégorie n'en découleClassification
machine, non validéePrédiction automatique; un appel candidat d’une seule tête enseignante, pas un consensus.
Le détail, modèle par modèle et score par score, se trouve en fin de page sous « Comment cette classification a été obtenue ».