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Jugular fossa meningioma: presentation and treatment options.

Abstract

Primary jugular fossa meningiomas are among the rarest subtypes of meningioma. They are intimately related to the lower cranial nerves, the jugular bulb and vein, and the temporal bone, and they have a tendency to extend both intra- and extracranially. The most common morbidity associated with jugular fossa lesions is lower cranial nerve deficits. In these cases, the differential diagnosis and preoperative radiographic diagnosis are very important because preoperative management and operative planning for the jugular fossa subtype differ considerably from those of other types of meningioma. Because of the rarity of this condition, our understanding of its treatment, long-term follow-up, and recurrence is limited. As experience with radiosurgical treatment of all meningiomas is accumulating, we may find that radiosurgery of jugular fossa meningiomas is appropriate. In the meantime, cure is still possible with complete surgical resection, and surgical morbidity can be minimized through meticulous planning and surgical technique. We describe a case of primary jugular fossa meningioma in a 45-year-old man who presented with complaints of chronic left aural fullness, hearing loss, and difficulty understanding voices. Imaging revealed the presence of a destructive jugular fossa mass. The patient underwent surgical resection without complication, and he was free of recurrence at i year of follow-up.

Introduction

Primary jugular fossa meningiomas are among the rarest of meningiomas. A particular patient's symptoms, signs, and treatment modalities are dependent on the relationship of the lesion to the lower cranial nerves, the jugular bulb and vein, and the temporal bone, and on whether the lesion has extended intra- or extracranially. Because jugular fossa meningiomas are so rare, our experience with their treatment, long-term follow-up, and recurrence is limited. Surgical removal of these lesions usually requires a skull base approach. The main surgical challenge is to preserve function in the lower cranial nerves, particularly in cranial nerves IX and X. Radiosurgical treatment of meningiomas at other sites has gained favor, but there is no reported experience with radiosurgery for jugular fossa meningiomas.

In this article, we describe a new case of jugular fossa meningioma, and we review the literature on this rare entity.

Case report

A 45-year-old man presented for evaluation of dysphonia and dysphagia. He had a 4- to 5-month history of aural fullness and decreased hearing in the left ear, and a 1-month history of difficulty understanding voices with his left ear.

Otoscopic examination revealed retraction of the left tympanic membrane. The Weber test lateralized to the left. Air conduction was greater than bone conduction bilaterally. Audiometric testing revealed a mild bilateral high-frequency sensorineural hearing loss (worse on the left) and a low-frequency conductive component on the left. Speech discrimination scores were 100% bilaterally.

Computed tomography (CT) of the temporal bones detected a 12 x 14-mm enhancing mass with bone destruction in the left jugular foramen (figure 1). The mass had eroded the adjacent wall of the external auditory canal and extended into the area of the round window. The jugulocarotid spine and hypoglossal foramen were intact. Opacification of the mesotympanum and hypotympanum was seen. Soft tissue extended into the area of the oval window, but the ossicles were intact. Magnetic resonance imaging (MRI) of the temporal bones identified a 16-mm enhancing focus in the area of the left jugular bulb along with bilateral mastoid air cell opacification and left middle ear opacification. The tumor did not enhance on magnetic resonance angiography (MRA) or transfemoral cerebral angiography.

[FIGURE 1 OMITTED]

Despite reviews of these images by several radiologists, neurosurgeons, and the primary surgeon (R.T.S.), the nature of the tumor was unclear at the time of surgery. The procedure involved a left intact-wall tympanomastoidectomy, a facial recess approach to the middle ear that exposed the tumor (figure 2), a retrofacial approach to the jugular bulb and skull base, a resection of the intra- and extracranial components of the jugular bulb neoplasm via a transmastoid approach to the hypotympanum and jugular fossa, and placement of a lumbar drain. Surgical pathology revealed that the skull base tumor was a jugular fossa meningioma (figure 3).

Postoperatively, the patient continued to have a mild to moderate high-frequency sensorineural hearing loss on the left, but his speech discrimination remained good and he experienced no surgical morbidity. His lower cranial nerves remained intact. MRI 1 year after surgery found no residual mass. The patient continues to be followed with an annual MRI and audiometry.

Discussion

Primary jugular fossa meningiomas arise from arachnoid cells that line the jugular bulb in the jugular fossa. (1) In many of the reported cases, jugular fossa meningiomas mimicked glomus jugulare tumors and lower cranial nerve neuromas. Jugular fossa meningiomas have an intimate relationship with the lower cranial nerves, the jugular bulb and vein, and the temporal bone, and they have a tendency to extend intra- and extracranially. These features make patients' symptoms bothersome and diminish their quality of life. (2) With careful preoperative evaluation, planning, and tailoring of the surgical approach, complete tumor resection can be achieved with the expectation of a good clinical outcome. Radiation should be discussed as an alternative, but this choice has shortcomings in many cases because the diagnosis often remains unknown until surgical tissue sampling has been performed; also, malignancy may present with a similar appearance.

Lesions that originate in the jugular foramen or the area of the hypotympanum may be associated with pulsatile tinnitus, hearing loss, aural fullness, hoarseness, and dysphagia; less common signs and symptoms are vertigo, balance dysfunction, and facial nerve dysfunction. Lesions may cause dysfunction of cranial nerves IX through XII, and they may either spare or involve the cochleovestibular system. Otomicroscopic evaluation and neuroradiographic assessment with CT, MRI, MRA, or conventional angiography often will help establish the diagnosis, or at least narrow the differential. (3)

Although basal posterior fossa meningiomas are likely to grow into the jugular fossa through the jugular foramen, primary jugular fossa meningiomas are extremely rare. Early reports tended to include jugular fossa meningiomas in another rare subgroup: temporal bone meningioma. This subgroup also included intratympanic, attic, and hypotympanic meningiomas and those of the geniculate ganglion, the sulci of the greater and lesser petrosal nerves, the jugular foramen, the internal acoustic meatus, the mastoid process, and the petrous bone. (4-9) Jugular fossa meningiomas were later separated into their own subgroup.

[FIGURE 2 OMITTED]

The jugular fossa and jugular foramen are frequently confused for each other, and these two terms are sometimes used interchangeably. In fact, the jugular foramen is an opening in the skull that connects the posterior cranial fossa and the jugular fossa. This opening is formed by the jugular notches in the temporal and occipital bones. It assumes an oblique position, extending from the lateral aspect posteriorly toward the medial aspect anteriorly. The jugular fossa, which is usually on the inferior surface of the petrous portion of the temporal bone, is a deep depression of variable size. It communicates with the posterior cranial fossa via the jugular foramen. It houses the jugular bulb, which continues as the jugular vein inferiorly and the sigmoid sinus superiorly.

Jugular fossa meningiomas frequently invade bone, including the jugular spine and the jugular tubercle. They frequently produce hyperostosis and bone thickening, but usually without the bone erosion that glomus jugulare tumors display. Jugular fossa neuromas may mimic the appearance of meningiomas on both T1- and T2-weighted MRIs, although the enhancement typically displayed by meningiomas is considerably greater. (10)

By virtue of the tumor's involvement with the jugular fossa and beyond, as well as the patency and dominance of the jugular bulb, removal of the lesion usually requires a skull base approach. The procedure can be performed via a transjugular, suprajugular, or retrojugular approach, depending on the extension of the tumor. (2) The senior author (R.T.S.) opted for a transmastoid approach, which provides easy and direct access to the lesion with minimal morbidity. The procedure is performed through a postauricular incision, which provides safe access to any small intracranial components through the jugular foramen.

The major management issue with these tumors concerns the lower cranial nerves, which are vulnerable to injury along their intracranial, extracranial, and foraminal segments and which may be impaired not only by surgery but also by the tumor itself. (11) Like glomus jugulare tumors, meningiomas grow around the individual nerves. (2,8) They are often adherent to the nerves within the confines of the jugular foramen, which in some cases makes it impossible to perform a total tumor resection with preservation of neural integrity; in such cases, a subtotal resection may be a better option, possibly followed by stereotactic radiosurgery (SRS). The decision must be made on the basis of the preoperative function of the nerves, the patient's age and health status, and the intraoperative findings. (12) A subtotal resection may also be best for patients in whom there is substantial adherence of the tumor to the carotid artery, brainstem, or spinal cord. (13,14)

[FIGURE 3 OMITTED]

Radiosurgery for jugular fossa meningiomas can be considered, especially in patients with atypical or malignant features and in the rare situation in which a patient has a single functioning sigmoid sinus and jugular bulb. (2) SRS represents an appealing option for patients whose age or medical condition would preclude surgery and for those with tumor recurrence after previous surgery. It is a less attractive option when a definitive diagnosis has not been established. Kondziolka et al evaluated the long-term results of 85 patients with meningiomas (90% with a skull base location) who underwent gamma knife radiosurgery from 1987 through 1992. (15) Some 53% of these patients experienced a decrease in tumor size. Five patients underwent tumor resection after SRS because of tumor growth or persistent symptoms. Five others experienced new or worsened neurologic deficits 3 to 31 months after SRS; the deficits resolved completely in 2 of these patients.

Because meningiomas do not demonstrate a radiographic response immediately after SRS, the use of other imaging techniques to assess response has been investigated. Nicolato evaluated the prognostic role of somatostatin receptor scintigraphy with the indium-111-labeled somatostatin analogue octreotide in 12 patients with skull base meningiomas who had undergone gamma knife radiosurgery. (16) He found a statistically significant difference in the pre- and post-SRS values of somatostatin concentration, a finding that suggested a correlation between a decrease in somatostatin receptor concentration and the meningioma shrinkage seen on subsequent MRI scans. So skull base surgeons should continue to followprogress in radiosurgeryand regularly reassess its potential role in the management of jugular fossa meningiomas.

In conclusion, jugular fossa meningiomas represent a rare subgroup of skull base tumor. With a thorough surgical resection, postoperative morbidity can be low, and in some cases, cranial nerve function can be preserved. Because of the rarity of jugular fossa meningiomas, it seems valuable to assess as much pooled information as possible through case reports and multicenter studies.

References

(1.) Molony TB, Brackmann DE, Lo WW. Meningiomas of the jugular foramen. Otolaryngol Head Neck Surg 1992;106(2):128-36.

(2.) Arnautovic KI, Al-Mefty O. Primary meningiomas of the jugular fossa. J Neurosurg 2002;97(1):12-20.

(3.) Pensak ML, Jackler RK. Removal of jugular foramen tumors: The fallopian bridge technique. Otolaryngol Head Neck Surg 1997;117 (6):586-91.

(4.) Buehrle R, Goodman WS,Wortzman G. Meningioma of the temporal bone. Can J Otolaryngol 1972;1:16-20.

(5.) Guzowski J, Paparella MM, Nageswara K, Hoshino T. Meningiomas of the temporal bone. Laryngoscope 1976;86(8):1141-6.

(6.) Maniglia AJ. Intra and extracranial meningiomas involving the temporal bone. Laryngoscope 1978;88(9 Pt 2 Suppl 12):1-58.

(7.) Maniglia AJ, Page LK. Posterior cranial fossa and temporal bone meningioma in a child, appearing as a neck mass. Otolaryngol Head Neck Surg 1979;87(5):578-83.

(8.) Nager GT, Heroy J, Hoeplinger M. Meningiomas invading the temporal bone with extension to the neck. Am J Otolaryngul 1983;4 (5):297-324.

(9.) Rietz DR, Ford CN, Kurtycz DF, et al. Significance of apparent intratympanic meningiomas. Laryngoscope 1983;93(11 Pt 1): 1397-1404.

(10.) Samii M, Babu RP, Tatagiba M, Sepehrnia A. Surgical treatment of jugular foramen schwannomas. J Neurosurg 1995;82(6):924-32.

(11.) Lustig LR, Jackler RK. The variable relationship between the lower cranial nerves and jugular foramen tumors: Implications for neural preservation. Am J Otol 1996;17(4):658-68.

(12.) Oghalai JS, Leung MK, Jackler RK, McDermott MW. Transjugular craniotomy for the management of jugular foramen tumors with intracranial extension. Otol Neuroto12004;25(4) :570-9; discussion 579.

(13.) Anand VK, Leonetti JP, Al-Mefty O. Neurovascular considerations in surgery of glomus tumors with intracranial extensions. Laryngoscope 1993;103(7):722-8.

(14.) Andrews JC, Valavanis A, Fisch U. Management of the internal carotid artery in surgery of the skull base. Laryngoscope 1989; 99(12):1224-9.

(15.) Kondziolka D, Nathoo N, Flickinger JC, et al. Long-term results after radiosurgery for benign intracranial tumors. Neurosurgery 2003;53(4):815-21; discussion 821-2.

(16.) Nicolato A. [sup.111]Indium-octreotide brain scintigraphy: A prognostic factor in skull base meningiomas treated with gamma knife radiosurgery. Q J Nucl Med Mol Imaging 2004;48(1):26-32.

Amy L. Rutt, DO; Xiaoli Chen, MD; Robert T. Sataloff, MD, DMA, FACS

From the Department of Otolaryngology-Head and Neck Surgery, Sinai-Huron Valley Hospital, Detroit Medical Center/Wayne State University, Detroit (Dr. Putt); and the Department of Pathology (Dr. Chen) and the Department of Otolaryngology-Head and Neck Surgery (Dr. Sataloff), Drexel University College of Medicine, Philadelphia.

Corresponding author: Robert T. Sataloff, MD, Department of Otolaryngology-Head and Neck Surgery, Drexel University College of Medicine, 1721 Pine St., Philadelphia, PA 19107. Phone: (215) 732-6100; fax: (215) 790-9359; e-mail: rtsataloff@phillyent.com
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Title Annotation:ORIGINAL ARTICLE
Author:Rutt, Amy L.; Chen, Xiaoli; Sataloff, Robert T.
Publication:Ear, Nose and Throat Journal
Article Type:Case study
Geographic Code:1USA
Date:Oct 1, 2009
Words:2255
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