Endolymphatic sac tumors: report of three cases.

Abstract:

OBJECTIVE AND IMPORTANCE:We present three cases of endolymphatic sac tumors and review the previously published cases. Despite frequent extension to the cerebellopontine angle, these rare tumors have only recently been recognized by neurosurgeons. CLINICAL PRESENTATION:A 26-year-old man developed a progressive hearing loss, revealing an intrapetrous retrolabyrinthine tumor on the right side. A 28-year-old woman experienced a left cerebellopontine angle syndrome, with a lytic intrapetrous mass extending into the cerebellopontine angle. A 38-year-old woman presented with an intracranial hypertension syndrome caused by a tumor of the jugular foramen. INTERVENTION:For the first and second patients, the tumors originated from the operculum of the endolymphatic sac. Total removal was achieved, via a transpetrosal approach, in these two cases. No recurrence was detected after a 20-month follow-up period. For the third patient, the tumor originated from the distal part of the sac. Recurrence was observed 8 years after subtotal removal via a retrosigmoid route. Histological analysis revealed a papillary-cystic adenocarcinomatous pattern in all cases, without features of aggressiveness. CONCLUSION:Endolymphatic sac tumors are locally invasive neoplasms characterized by bipolar intrapetrous and posterior fossa involvement. The anatomic complexity of the endolymphatic sac may explain the distinct patterns of extension of these tumors. Early radical surgery is related to good outcomes.

journal_name

Neurosurgery

journal_title

Neurosurgery

authors

Roche PH,Dufour H,Figarella-Branger D,Pellet W

doi

10.1097/00006123-199804000-00139

subject

Has Abstract

pub_date

1998-04-01 00:00:00

pages

927-32

issue

4

eissn

0148-396X

issn

1524-4040

journal_volume

42

pub_type

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