论文信息 - Deafness duetobilateral endolymphatic sac tumoursina caseofvon Hippel-Lindau syndrome

Deafness duetobilateral endolymphatic sac tumoursina caseofvon Hippel-Lindau syndrome

A caseofbilateral endolymphatic sac tumoursisreported. Inapatient withvon Hippel-Lindau syndrome, tumourgrowth intheright cerebellopontine anglecaused deafness. Thetumourwasremovedand classified asa metastasis fromathyroid carcinoma. However,on thyroidectomy no primaryneoplasmcouldbe found. Eightyearslatera similar tumourwas operated on intheleftpetrosal bone. Histological appearance, inumunocytochemical findings, andtheclinical contextgaveevidence thatthetumourshad tobe reclassified asendolymphatic sac tumoursxtremelyrareentities. The reportsupports thehypothesis, suggested by thefew earlier casereports, that endolymphatic sactumourscouldbeone oftheinherent tumourmanifestations in vonHippel-Lindau syndrome. (3Neurol Neurosurg Psychiatry 1996;61:318-320)

G. Kempermann | W. Mann | R. Laszig | R. Scheremet

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