Simultaneous contralateral vestibular schwannoma and glomus jugulare tumor: a case report.

Abstract

To the best of our knowledge, only 3 cases of a simultaneous vestibular schwannoma and a glomus jugulare tumor have been previously reported in the literature. In all 3 cases, the lesions were located on the same side. We report a new case of simultaneous vestibular schwannoma and glomus jugulare tumor that is unique in that the two lesions arose on opposite sides. The glomus tumor was treated with embolization followed by radiotherapy, while the schwannoma was managed via radiologic observation.

Introduction

Vestibular schwannomas arise from Schwann cells on branches of the vestibulocochlear nerve, most commonly the superior vestibular nerve. The annual incidence is 1 per 100,000 population. (1) Glomus jugulare tumors 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. The annual incidence is 1 in 1.3 million. (2)

To the best of our knowledge, only 3 cases of a simultaneous vestibular schwannoma and a glomus jugulare tumor have been previously reported in the literature. (3-5) In all 3 cases, the lesions were located on the same side (table). In this article, we report a new case of simultaneous vestibular schwannoma and glomus jugulare tumor that is unique because the two lesions arose on opposite sides.

Case report

A 66-year-old woman presented with a 6-year history of right-sided hearing loss and a 2-year history of left-sided pulsatile tinnitus. Other symptoms included unsteadiness and left-sided headaches. Her medical history included right-sided chronic suppurative otitis media.

Otoscopy revealed the presence of a central perforation of the right tympanic membrane and a red pulsatile mass behind the left tympanic membrane. Neurologic examination demonstrated normal cranial nerves and cerebellar function. The results of Romberg's test were negative and findings on Unterberger's stepping test were normal, but the patient was unsteady on tandem gait with her eyes open.

Pure-tone audiometry revealed a mixed hearing loss in both ears, with air conduction thresholds averaging 82 dB in the right ear and 44 dB in the left. Speech audiometry showed a maximum discrimination score of 0% at 80 dB in the right ear (representing socially useless hearing) and 100% at 80 dB in the left ear. Magnetic resonance imaging (MRI) detected a l5-mm (maximum mediolateral diameter) vestibular schwannoma on the right (figure 1, A), and computed tomography (CT) identified a Fisch type D glomus jugulare tumor on the left (figure 1, B). Carotid angiography demonstrated characteristics typical of a large glomus tumor that was supplied primarily by branches of the external carotid artery (figure 2).

[FIGURES 1-2 OMITTED]

The glomus tumor was treated with embolization followed by radiotherapy; the schwannoma was left alone under radiologic observation. The patient was assessed annually with neurotologic examinations and MRIs. She remained stable and exhibited no evidence of any change in the size of the schwannoma.

Discussion

In the previously reported cases of simultaneous vestibular schwannoma and a glomus jugulare tumor, which occurred in women aged 65 to 79 years, all 3 patients presented with symptoms in one ear only. (3-5) A vestibular schwannoma was diagnosed incidentally on radiologic investigation for a glomus jugulare tumor in 2 cases, (4,5) and a glomus jugulare tumor was diagnosed incidentally at operation for a vestibular schwannoma in 1 case. (3) In contrast, our patient presented with symptoms in both ears, and the vestibular schwannoma and glomus jugulare tumor were diagnosed simultaneously on radiologic investigation.

Management options for both vestibular schwannomas and glomus jugulare tumors include observation, surgery, and radiotherapy. The 3 previous cases were all managed differently--with surgery, (3) observation, (4) and radiotherapy. (5) In each case, management was straightforward because the two tumors were ipsilateral.

Because the lesions in our patient were contralateral, management was not as straightforward. With a 15-mm vestibular schwannoma in a right ear with no useful hearing and a Fisch type D glomus jugulare tumor in a left ear with some hearing, our priority was to manage the disease in both ears while preserving the hearing on the left. In view of its size, the glomus jugulare tumor on the left clearly required treatment, but because the vestibular schwannoma was relatively small, we adopted a watch, wait, and rescan policy after careful consideration. We decided that embolization followed by radiotherapy was preferable to surgery because this would be likely to control the disease without the need for major surgery, and it would not put the available hearing at as much risk.

Table. Characteristics of the 4 reported cases of simultaneous
vestibular schwannoma and glomus tumor

                                         Schwannoma
                               Age/
Author                         sex     Side      Size

Weber et a1, (3) 1992          65/F    Left     20 mm
Falcioni et al, (4) 1998       72/F    Left      5 mm
Schallawitz et al, (5) 2001    79/F    Right    12 mm
Moumoulidis et al, * 2005      66/F    Right    15 mm

                                  Glomus tumor

Author                         Side    Size           Management

Weber et a1, (3) 1992          Left    Fisch type A   Surgery
Falcioni et al, (4) 1998       Left    Fisch type A   Conservative
Schallawitz et al, (5) 2001    Right   Fisch type C   Radiotherapy
Moumoulidis et al, * 2005      Left    Fisch type D   Schwannoma:
                                                      observation;
                                                      glomus tumor:
                                                      embolization,
                                                      radiotherapy
* Present case.

References

(1.) Tos M, Thomsen J. Epidemiology of acoustic neuromas. J Laryngol Otol 1984;98:685-92.

(2.) Moffat DA, Hardy DG. Surgical management of large glomus jugulare tumours: Infra- and trans-temporal approach. J Laryngol Otol 1989;103:1167-80.

(3.) Weber R, Draf W, Bachor E. [Simultaneous occurrence of an acoustic neurinoma and a glomus tympanicum tumor in a patient. An unusual constellation and problematic surgical task]. HNO 1992;40:410-13.

(4.) Falcioni M, Caruso A, Sanna M. [Coexistence of vestibular schwannoma and glomus tympanicum tumor]. Acta Otorhinolaryngoltal 1998;18:398-401.

(5.) Schallawitz T, Strauss G, Bootz F. [Pulsating space-occupying lesion of the right tympanum after radical operation. Glomus jugulare tumor and acoustic neuroma right]. HNO 2001;49:226-7.

From the Department of Otolaryngology, University of Cambridge, Addenbrooke's Hospital, Cambridge, U.K.

Reprint requests: Mr. Ioannis Moumoulidis, 36 Moorhouse Way, Kettering, Northants, NN15 7LX, UK. Phone: [phone]; e-mail: [e-mail]