Tu. Cerebrale III

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  TUMORI CEREBRALE III  Neurofibromatoza (tumori neurogenice) ã Neurofibromatoza tip I (b.von Recklinghausen) ã - transmitere ereditara autozomal dominanta-cromozomul17 (50%) ã -aparitie sporadica 50% ã -incidenta: 1 : 2000-6000 nascuti vii ã -varsta: copilarie Diagnosticul -doua sau mai multe din: ã 1.sase sau mai multe pete cutanate cu aspect “cafea cu lapte“ã 2.doua sau mai multe hamartoame in iris ã 3.doua sau mai multe neurofibroame sau unul sau mai multe neurofibroame plexiforme ã 4. pistrui inghinali sau axilari ã 5. displazie osoasa pe unul sau mai multe oase sau pseudartroza pe un os lung ã 6. gliom de nerv optic (astrocitom pilocitic de n. optic sau cai optice) ã 7. o ruda de grad I cu NF tip I  Neurofibromatoza (tumori neurogenice) ã Neurofibromatoza tip I (b.von Recklinghausen) ã - 38% din cei cu gliom de nerv optic au NF -I ã -15-40% din cei cu NF-I au gliom de nerv optic ã  Neurofibroamele degenereaza sarcomatos in perioada adulta ã  Neurofibromul plexiform are rata de malignizare crescuta ã Asociaza meningocele (ectazie durala in canalul spinal) ã +/-stenoza de apeduct Sylvius ã  pahigiria, ã  poilimicrogiria, ã heterotopii, ã siringomielie  Neurofibromatoza (tumori neurogenice) ã Neurofibromatoza tip II (NF-II) ã Incidenta: NF-II/NF-I 1:10Diagnosticul  –  una din: 1.Neurinom de acustic bilateral2.Neurinom de acustic unilateral + o ruda de grad I cu NF-II 3.Doua din: neurinom, neurofibrom, meningeom sau gliom 4.Cataracta posterioara sublenticulara capsulara + pacient tanar 5.Neurinom de nerv trigemen (a doua localizare) Tumori asociate mai frecvent:MISME = Multiple Inherited Schwanoma Meningeoma Ependimoma
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