CASE REPORTS   

Journal of Neurosurgical Sciences 2001 September;45(3):177-80

Copyright © 2001 EDIZIONI MINERVA MEDICA

language: English

Endolymphatic sac tumour. A case report

Nestler U. ¹, Winking M. ¹, Huegens-Penzel M. ², Kuchelmeister K. ³, Boeker D. K. ¹

¹ Department of Neurosurgery, Justus Liebig-Universität, Giessen, Germany; ² Department of Neuroradiology, Justus Liebig-Universität, Giessen, Germany; ³ Institute of Neuropathology, Justus Liebig-Universität, Giessen, Germany

We ­present the ­case of a ­patient ­with an endo­lym­phat­ic sac ­tumour (­ELST). This ­rare ­tumour ­entity has ­only recent­ly ­been ­defined and ­despite a ­well char­ac­ter­ized clin­i­cal appear­ance mis­di­ag­no­sis as jug­u­lar par­a­gan­gli­o­ma is fre­quent. A 68-­year-old wom­an was admit­ted to our Neu­ro­sur­gi­cal Depart­ment ­with a ­mass ­lesion extend­ing ­from the ­left tem­po­ral ­bone to the cer­e­bel­lo-pon­tine ­angle (CPA). Radiological fea­tures ­were ­high vas­cu­lar­iza­tion, con­trast enhance­ment and destruc­tion of the os petro­sum. After pre­op­er­a­tive angio­graph­ic embol­iza­tion the ­tumour was ­removed in two sur­gi­cal inter­ven­tions, ­first via a lat­er­al sub­oc­cip­i­tal ­approach and sec­ond by pet­ro­sec­to­my. The ­lesion ­proved to be a typ­i­cal endo­lym­phat­ic sac ­tumour by a syn­op­sis of his­to­log­i­cal, radio­log­i­cal and clin­i­cal fea­tures. ­ELST ­should be tak­en ­into con­sid­er­a­tion in ­patients ­with ­mass ­lesions in the cer­e­bel­lo­pon­tine ­angle destroy­ing the ­petrous ­bone and resem­bling par­a­gan­gli­o­ma. Since ­slow ­growth ­rate and ­lack of metas­ta­ses are par­tic­u­lar fea­tures of ­ELST, com­plete resec­tion of the ­tumour ­results in ­long sur­vi­val ­times with­out adju­vant ­chemo- or radio­ther­a­py.