Endolymphatic sac tumours: surgical management.
BACKGROUND
Endolymphatic sac tumours (ELSTs) have been known as an individual tumour entity only since 1984. ELSTs may occur either solitarily and sporadically or as a hereditary manifestation associated with von Hippel-Lindau (VHL) disease. The latter association was first observed in 1992 and confirmed by molecular genetic analysis of the VHL gene. No consensual diagnostic and treatment strategy of ELST exists at present.


METHODS
Based on imaging criteria in computed tomography, magnetic resonance imaging (MRI), and magnetic resonance angiography, we developed a staging system to classify ELST in a series of seven consecutive patients in an attempt to custom-tailor the surgical approach. Type A referred to tumours that were locally confined without temporal bone erosion or infiltration of the dura (n = 2); type B tumours showed evidence of bone infiltration of the osseous labyrinth and sensorineural hearing loss (n = 2); and in type C, the tumour further invaded the sigmoid sinus and jugular bulb (n = 3). Two patients suffered from VHL disease.


RESULTS
In all patients, the tumour was completely removed. Stage-adapted surgical approaches included various transpetrosal procedures, from the translabyrinthine to the infratemporal approaches. The functional integrity of the facial nerve was maintained in all tumour stages, whereas the vestibulocochlear nerve could be preserved only in patients with type A tumours. Follow-up MRI demonstrated no local tumour recurrence during a postoperative observation period ranging from 4 to 38 months.


CONCLUSIONS
Stage-based surgical strategy enables the complete removal of ELST with minor morbidity. Transmastoid approaches are most efficient for resection of the tumour matrix to prevent local recurrence.
Schipper
Jörg
J
Department of Head and Neck Surgery, University of Freiburg, Freiburg, Germany. schipper@hno.ukl.uni-freiburg.de
Maier
Wolfgang
W
Rosahl
Steffen K
SK
van Velthoven
Vera
V
Berlis
Ansgar
A
Boedeker
Carsten Christof
CC
Laszig
Roland
R
Teszler
Christian Barna
CB
Ridder
Gerd Jürgen
GJ
D000328:Adult; D003952:Diagnostic Imaging; D004428:Ear Neoplasms / Q000473:pathology* / Q000601:surgery*; D004712:Endolymphatic Sac; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D009361:Neoplasm Invasiveness; D009367:Neoplasm Staging / Q000379:methods*; D013506:Otologic Surgical Procedures / Q000379:methods*; D012189:Retrospective Studies; D016896:Treatment Outcome