D.GarethR.EvansandStavrosM.Stivaros

IntheirarticleinthecurrenteditionofthejournalAsthagiriandcolleagues1elegantlydemonstratethatthelobulatedappearanceofvestibularschwannomasinNF2isduetothemultifocaloriginofseparateclonaltumorsthathavedistinctsecondsomatic‘hits’intheNF2gene.InKnudson2publishedthe“twohit”hypothesisoftumorigenesisforretinoblastomawithbothcopiesofthegeneneedingtobeinactivatedtoinitiatetumorigenesis.Heexplainedthatthepresenceofagermlinemutationmeantthattumorswerehighlylikelytobebilateralandmultifocalasopposedtoisolatedinthosewithasomaticorigintoboth‘hits’.NF2isperhapsthebestexampleofthismechanism,otherthanretinoblastomaasclearlydemonstratedinthisissue.

Evenwithimprovementsinmicro-surgeryandwithuseofradiationtherapy,thegreatmajorityofindividualswithNF2be







































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