Extracranial Meningioma with Middle Ear Invasion Via the Jugular Foramen: A Case Report

Introduction: Extracranial meningiomas are an extremely rare entity, accounting for less than 1% of all meningiomas.¹ Involvement of the ear and temporal bone is an even rarer clinical subset, with less than 100 cases reported in the literature and no definitive treatment paradigm recommended.²

Case Description: We present the case of a 42-year-old female who presented with a chief complaint of 2 years of gradual left sided hearing loss and intermittent acute otitis media. Prior to presentation at our clinic, she had undergone outside evaluation with preliminary diagnosis of cholesteatoma with recommendation of surgical removed. This was delayed and, prior to presentation to our clinic, the patient had noted development of pulsatile tinnitus and dysphagia. Audiometric testing demonstrated evidence of progressive conductive hearing loss. Initial imaging studies revealed a lateral skull base mass involving the left jugular foramen, middle ear and cerebellopontine angle. Differential favored jugulotympanic paraganglioma versus meningioma.

Management and Outcome: This patient’s case was discussed at our Skull Base Tumor Board and given the potential morbidity of infratemporal fossa approach and a very low probability for complete resection, the decision was made to proceed with left tympanoplasty, middle ear exploration and debulking of the tumor. Intraoperatively, the tumor was noted to be extending to the Eustachian tube orifice, the ossicles, and down to the jugular bulb in the hypotympanum. Pathologic specimen confirmed a WHO Grade I Meningioma. Adjuvant treatment was performed with 54 Gy/30 fraction proton therapy. At last follow-up, she was thirty-seven months post completion of adjuvant therapy with stable disease and resolution of her left sided hearing loss and pulsatile tinnitus.

Discussion: We present a rare case and long-term outcomes of an extratemporal meningioma involving the jugular foramen, middle ear and cerebellopontine angle, managed with debulking and proton beam therapy. Choice of treatment in these cases is dictated by proximity to critical structures and, therefore, associated risk of morbidity with total surgical resection. A multimodal treatment approach with tumor debulking and adjuvant particle therapy has been shown in limited numbers to be an effective treatment modality for skull base meningioma with low complication rates and improvements in quality of life.³

Conclusion: Herein, we present a case of extracranial meningioma involving multiple critical structures of the lateral skull base, which was successfully managed with debulking and adjuvant proton therapy now 3 years following treatment. This adds to the limited body of literature regarding management of this rare presentation, which we plan on expanding to a future case series.

 

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Artikel online veröffentlicht:
05. Februar 2024