The endolymphatic sac tumor: challenges in the eradication of a localized disease
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OTOLOGY
The endolymphatic sac tumor: challenges in the eradication of a localized disease Vittoria Sykopetrites1 · Gianluca Piras1 · Annalisa Giannuzzi1 · Antonio Caruso1 · Abdelkader Taibah1 · Mario Sanna1 Received: 16 June 2020 / Accepted: 24 August 2020 © Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Objective Identify the critical points that lead to recurrences and lack of radicality in endolymphatic sac tumors (ELSTs). Study design Retrospective case study and review of the literature. Setting Tertiary referral center. Patients Thirteen cases of ELST were included in the study and their preoperative, intraoperative and postoperative data were analyzed and compared to a review of the literature. Intervention(s) Therapeutical. Main Outcome Measure(s) Prevalence of recurrent and residual tumors, comparison to the literature and analysis of ELST characteristics. Results Diagnosis was made 26 ± 17 months after the onset of symptomatology, and an ELST was preoperatively suspected in only six cases. At the time of surgery, 10 patients suffered from hearing loss. Preoperative symptoms or audiometry could not predict labyrinth infiltration, although speech discrimination scores were significantly associated with labyrinth infiltration (p = 0.0413). The labyrinth was infiltrated in 8 cases (57.1%), and in 7 cases (46.7%) the tumor eroded the carotid canal, whereas 6 cases (40%) presented an intradural extension. A gross total resection was achieved in 11 cases. There were two residual tumors, one of which because of profuse bleeding, and one recurrence (23.1%). A mean of 22.8% of recurrent or residual tumors are described in the literature based on 242 published cases, in more than half of the cases as a consequence of subtotal tumor resection (STR). Conclusions Recurrence derives mostly from the difficulty to identify the extension of the tumor due to the extensive bone infiltration. Accurate diagnosis and correct preoperative planning, with embolization when possible, will facilitate surgery and avoid STR due to intraoperative bleeding. Long follow-ups are important in order to avoid insidious recurrences. Keywords Endolymphatic sac tumor · Von hippel-lindau disease · Low-grade adenocarcinoma · Temporal bone tumor
Introduction The anatomy, physiology and pathophysiology of the endolymphatic sac (ELS) have fascinated scientists for centuries, its complete purpose still remains vague, and particular functions are being unveiled only as a result of the diseases that affect it [1].
* Vittoria Sykopetrites [email protected] 1
Department of Otology and Skull Base Surgery Gruppo, Otologico and Mario Sanna Foundation, Casa Di Cura “Piacenza” S.P.A, Piacenza‑RomePiacenza, Italy
Similarly, the ELS tumor (ELST) has been largely neglected as a distinct entity, up until 1989, when Heffner described a series of 20 cases of a “low-grade papillary adenocarcinoma of the temporal bone of probable ELS origin” [2]. Finally, in 2005, the World Health Organization Head and Neck tumor classif
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