title: Management of primary and recurrent endolymphatic sac tumors.
abstract: OBJECTIVE
  To report the clinical presentation, management strategies, and outcomes of 14 endolymphatic sac tumors (EST).
  
  
  METHODS
  Retrospective case series.
  
  
  METHODS
  Collective experience accrued from 2 tertiary referral centers.
  
  
  METHODS
  All patients with ESTs.
  
  
  METHODS
  Microsurgery, stereotactic radiosurgery (SRS).
  
  
  METHODS
  Treatment-related morbidity, recurrence.
  
  
  RESULTS
  Fourteen ESTs (13 patients) met inclusion criteria. Eight tumors were sporadic, and 6 were associated with von Hippel-Lindau disease (VHL). Twelve lesions were managed primarily, whereas 2 were referred for treatment of sizable recurrences after subtotal resection. Including all patients, the median age at diagnosis was 36 years, and the median duration of posttreatment follow-up was 78 months. Among primary tumors, the median delay between symptom onset and diagnosis was 36 months, and the median tumor diameter at presentation was 23 mm with most lesions demonstrating intracranial involvement. Subjects with VHL frequently presented with smaller, less extensive tumors and were more commonly female compared with patients with sporadic disease. Of the 9 ESTs presenting with normal facial nerve function, 8 maintained good (HB 1-2) posttreatment capacity. Two of 5 ears with useful preoperative hearing maintained pretreatment hearing levels after surgery. One of 12 tumors managed primarily recurred after microsurgical resection. Primary SRS was used in 1 medically infirm patient providing durable tumor control (94 mo) at last follow-up. Among recurrent tumors, 2 were managed successfully with reexcision, whereas 1 underwent salvage surgery followed by 2 sequential treatments of SRS for 2 separate intracranial recurrences; no in-field recurrence has been detected to date.
  
  
  CONCLUSIONS
  ESTs are rare primary neoplasms of the temporal bone that may occur sporadically or in association with VHL. Outcomes after treatment of small less-extensive tumors are favorable compared with patients with advanced primary or recurrent disease. Furthermore, subtotal resection carries a high risk of bulky or multifocal recurrence. As such, early gross total resection remains the management strategy of choice for primary and recurrent ESTs; however, SRS should be considered in poor surgical candidates or in cases of focal intracranial recurrence when the morbidity of salvage surgery is high.
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Mayo Clinic School of Medicine, Rochester, Minnesota, USA.
authors.forename: Matthew L
authors.initials: ML
authors.lastname: Carlson
authors.forename: Joshua J
authors.initials: JJ
authors.lastname: Thom
authors.forename: Colin L
authors.initials: CL
authors.lastname: Driscoll
authors.forename: David S
authors.initials: DS
authors.lastname: Haynes
authors.forename: Brian A
authors.initials: BA
authors.lastname: Neff
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Link
authors.forename: George B
authors.initials: GB
authors.lastname: Wanna
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D004428:Ear Neoplasms / Q000473:pathology* / Q000601:surgery*; D004712:Endolymphatic Sac / Q000473:pathology* / Q000601:surgery*; D005260:Female; D006801:Humans; D008297:Male; D008866:Microsurgery / Q000379:methods; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D016634:Radiosurgery / Q000379:methods; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult