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Simultaneous Contralateral Vestibular Schwannoma and Middle Ear Paraganglioma Tumor.

INTRODUCTION

Vestibular schwannomas (VS) arise from the Schwann cells on the branches of the vestibulocochlear nerve, most commonly the superior vestibular nerve. The incidence is 2 in 10,000 [1]. VS account for approximately 6% of all intracranial tumors [2]. Paragangliomas arise from glomus bodies or paraganglionic tissue in the adventitia of the jugular bulb, usually in relation to the auricular branches of the vagus and glossopharyngeal nerves. MEPs are rare tumors, representing less than 0.5% of all head and neck tumors. Approximately, 3% of them occur in the head and neck area; other estimates suggest that they are 1 in 30,000 head and neck tumors [3]. To the best of our knowledge, only 5 cases of simultaneous VS and MEP have been previously reported in literature. In the first 3 cases the lesions were located on the same side, but in the fourth and fifth they were located contralaterally (Table 1). Here, we report a new case of simultaneous contralateral VS and MEP and present a review of the literature.

CASE PRESENTATION

A 43-year-old male presented with an 11-year history of right-sided hearing loss and dizziness and a 1-year history of left-sided pulsatile tinnitus. Speech audiometry showed a maximum discrimination score on the right side of 30% at 80 dB (Figure 1). Otoscopy revealed the presence of a red pulsatile mass behind the left tympanic membrane (Figure 2a). Pure-tone audiometry revealed sensorineural, down-sloping hearing loss on the right side and low-tone conductive hearing loss on the left side. The patient underwent an axial high-resolution computed tomography (HRCT), which showed a 4.9 mm round lesion of the same density as soft tissues adherent to the promontory wall of the left ear (Figure 2b). Turbo spin echo magnetic resonance imaging (MRI) detected a hyper-intense nucleus at the base of the basilar turn of the cochlea (Figure 2c) of the left ear on T2 in the coronal image. A T1-weighted three-dimensional gradient-echo technique with gadolinium injection showed a marked increase of the signal at the level of the lesion in the hypotympanum in contact with the promontory wall. When analyzing the lesion using a dynamic sequence turbo fast field echo, an enhancement in the arterial phase was found during gadolinium injection, permitting the identification of the lesion as a 4.9 mm type A1 MEP of the left temporal bone (Figure 2d-f). T1 images with gadolinium also showed a 17 mm VS on the right temporal bone, which did not reach the fundus of the internal auditory canal (Figure 3a). The right VS was removed with a translabyrinthine approach that resulted in total hearing loss on the same side. For this reason, it was temporarily decided not to treat the MEP surgically, but to observe it with planned radiological examinations. After 1 year, an exacerbation of pulsatile tinnitus was reported by the patient and both HRCT and MRI were repeated. The images showed that the MEP had increased in size from 4.9 mm to 6.2 mm (Figure 3b, c), thereby becominga type A2 paraganglioma [4]. Because of the worsening of symptoms (tinnitus) and more importantly, because of tumor growth, after an appropriate and thorough counselling with the patient, the MEP was surgically removed via a retroauricular, laser-assisted approach. Both the preoperative diagnoses were confirmed by pathologic examination of the specimens. One month after surgery, the patient reported disappearance of pulsatile tinnitus with preservation of the preoperative auditory threshold. All the described diagnostic and therapeutical procedures were performed after obtaining a signed informed consent by the patient.

DISCUSSION

To our knowledge, only 5 cases of simultaneous VS and MEP have been reported in literature in the same patient: 3 cases being localized on the same temporal bone and the other 2 cases in the contralateral ear. As in the present case, the therapeutic approach for such a situation could be controversial, considering many treatment possibilities for both lesions, including observation, radiotherapy, and surgery. Moumoulidis et al. [5] described the first case of simultaneous VS and MEP affecting both sides and proposed a wait-and-scan policy for hearing preservation purposes also due to the limited size of the VS, while the larger paraganglioma was treated with embolization and radiotherapy. The authors chose these approaches instead of surgery for a safer hearing control [5]. Ceylan et al. [6] reported on a 63-year-old woman presenting with a 22 mm VS on the right side, which they decided to treat with gamma knife stereotactic radiosurgery, while the left-sided type D paraganglioma was treated with embolization and radiotherapy (Table 1). These two different approaches were therefore motivated by the relatively large dimensions of both lesions and by the intention of hearing preservation.

In the present report, both lesions were relatively small initially. The young age of the patient was the main motivation for the choice to first treat the VS surgically via a translabyrinthine approach due to the poor speech discrimination in the affected ear. Due to the resulting total hearing loss, it was at first decided not to treat the GJT on the left side, but to wait and scan it regularly. Due to the worsening of symptoms (pulsatile tinnitus) and to lesion growth, surgery was instead planned 2 years later to prevent possible complications in the event of additional growth. The decision to perform surgery on GJT was consented by the patient, explaining the possible postoperative hearing issues. Surgery was performed through a retroauricular approach, with complete lesion removal and no effects on residual hearing.

One month after surgery, the patient reported the disappearance of pulsatile tinnitus without deterioration of hearing in the left ear. If surgical removal of the glomus had led to a worsening auditory threshold of the left ear, a conventional or semi-implantable hearing aid would have been recommended.

CONCLUSION

The concomitant presence of tumoral lesions affecting both temporal bones deserves caution, as far as their treatment is concerned, due to the likelihood of bilateral hearing damage. The decision of a surgical removal should thoroughly be discussed with the patient in accordance with the clinical behaviour of these lesions.

Informed Consent: Written informed consent was obtained from the patient who participated in this study.

Peer-review: Externally peer-reviewed.

Author contributions: Concept - E.C., M.B., L.V; Design - E.C., M.B., L.V. Supervision - M.B, S.M; Resource - G.T., L.V.; Materials - G.T., L.V.; Data Collection and/or Processing - G.T., E.C., L.V, C.F., V.F.; Analysis and/or Interpretation - M.B., G.T.; Literature Search - C.F., V.F.; Writing - E.C., M.B., S.M.; Critical Reviews - S.M., M.B.

Conflict of Interest: No conflict of interest was declared by the authors.

Financial Disclosure: The authors declared that this study had received no financial support.

REFERENCES

[1.] Lin D, Hegarty JL, Fischbein NJ, Jackler RK. The prevalence of "incidental" acoustic neuroma. Arch Otolaryngol Head Neck Surg 2005; 131: 214-4. [CrossRef]

[2.] Howitz MF, Johansen C, Tos M, Charabi S, Olsen JH. Incidence of vestibular schwannoma in Denmark, 1977-1995. Am J Otol 2000; 21: 690-4.

[3.] Wasserman PG, Savargaonkar P. Paragangliomas: classification, pathology, and differential diagnosis. Otolaryngol Clin North Am 2001; 34: 845-62. [CrossRef]

[4.] Sanna M, Fois P, Pasanisi E, Russo A, Bacciu A. Middle ear and mastoid glomus tumors (glomus tympanicum): an algorithm for the surgical management. Auris Nasus Larynx 2010; 37: 661-8. [CrossRef]

[5.] Moumoulidis I, Brewis C, Moffat DA. Simultaneous contralateral vestibular schwannoma and glomus jugulare tumor: a case report. Ear Nose Throat J 2005; 84: 290-2.

[6.] Ceylan K, Emir, H, unlu I, Bayiz U. Simultaneous contralateral vestibular schwannoma and glomus jugulare. Mediterr J Otol 2007; 3: 95-8.

Edoardo Covelli, Guido Trasimeni, Chiara Filippi, Valerio Fabiani, Simonetta Monini, Luigi Volpini, Maurizio Barbara

Clinic of Otolaryngology, Medicine and Psychology, Sapienza Universita di Rome, Italy

Cite this article as: Covelli E, Trasimeni G, Filippi C, Fabiani V, Monini S, Volpini L, et al. Simultaneous Contralateral Vestibular Schwannoma and Middle Ear Paraganglioma Tumor. J Int Adv Otol 2017; 13: 426-9.

This study was presented at the 1st Congress of the Italian Society of Otology and Hearing Sciences, 22-25 September 2016, Ferrara, Italy.

Corresponding Address: Maurizio Barbara E-mail: maurizio.barbara@uniroma1.it

Submitted: 28.08.2017 * Accepted: 21.11.2017

DOI: 10.5152/iao.2017.4362
Table 1. Characteristics of the 3 cases of simultaneous contralateral
VS and Paraganglioma

Author                        Age/Sex       VS              GJT
Moumoulidis et al. [5] 2005  66/Female  Right 15 mm  Left Fisch type D

Ceylan et al. [6] 2007       63/Female  Right 22 mm  Left Fisch type C2

The present study            41/Male    Right 17 mm  Left Fisch type A2

Author                               Management
Moumoulidis et al. [5] 2005  Schwannoma observation
                             Glomus tumor embolization radiotherapy
Ceylan et al. [6] 2007       Schwannoma gamma knife stereotactic
                             Glomus tumor embolization radiotherapy
The present study            Schwannoma surgery
                             Glomus tumor surgery

VS: vestibular schwannoma
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Title Annotation:Case Report
Author:Covelli, Edoardo; Trasimeni, Guido; Filippi, Chiara; Fabiani, Valerio; Monini, Simonetta; Volpini, L
Publication:The Journal of the International Advanced Otology
Date:Dec 1, 2017
Words:1472
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