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Paraganglioma presenting as cholesterol granuloma of the petrous apex.

Abstract

We report the unique finding of a petrous apex cholesterol granuloma associated with a paraganglioma, also known as a glomus jugulare tumor, in a 52-year-old woman who presented to our department with pulsatile tinnitus, hearing loss, aural fullness, and disequilibrium. She had been treated for a petrous apex cholesterol granuloma 20 years earlier, at which time she had undergone drainage of the granuloma via subtotal petrous apicectomy. When she carne to our facility approximately 20 years later, she had signs and symptoms consistent with a jugular paraganglioma, which was likely to have been present at the time of her initial presentation for the cholesterol granuloma. In fact, microscopic bleeding from the paraganglioma might have led to the formation of the cholesterol granuloma. The metachronous presentation of these two entities, which to our knowledge has not been reported previously in the literature, indicates the potential association of paragangliomas with the formation of cholesterol granulomas of the petrous apex.

Introduction

A number of lesions and disease processes have been described within the petrous apex, including cholesterol granulomas, petrous apicitis, cerebrospinal fluid cysts, encephaloceles, epidermoids, effusions, mucoceles, carotid aneurysms, asymmetric pneumatization, chordomas, chondrosarcomas, metastases, paragangliomas (glomus tumors), meningiomas, and schwannomas, (1-8) Characteristic presenting symptoms, as well as findings on magnetic resonance imaging (MRI) and computed tomography (CT) scans, are essential for differentiation of the lesion. (1,2,9,10) For example, paragangliomas characteristically present as isointense lesions on T1-weighted MRI and isointense to hyperintense lesions on T2-weighted MRI; they show opacification with destruction of air cells on CT.

Cholesterol granulomas represent the most common lesion or disease process involving the petrous apex. They were first described in the 1980s. (11-13) Cholesterol granulomas are characterized by heterogeneous hyperintense lesions on T1- and T2-weighted MRI. (1,2,9,10) On CT, they present as opacifications within the petrous apex, with expansile bone destruction. (1,2,9,10)

The pathogenesis of petrous apex cholesterol granuloma remains controversial. Traditionally, it is thought to be an inflammatory reaction to cholesterol crystals released from blood breakdown products during anaerobic catabolism of blood and blood products. (14-19) The presence of blood and blood products is theorized to arise from transudative hemorrhage caused by negative pressure within the mucosa-lined air cells. (14-19) Jackler and Cho propose an alternative hypothesis: that blood products arise from dehiscences of the bony partition between the petrous apex air cell system and the bone marrow in the anterior temporal bone or clivus. (19) They suggest that the exposed marrow is a potential source of repeated hemorrhage. (19)

This article describes the presentation of a cholesterol granuloma in a patient with a paraganglioma, or glomus jugulare tumor. It is proposed that the paraganglioma might have been the source of recurrent hemorrhage, leading to the formation of the cholesterol granuloma.

Case report

A 52-year-old woman presented to our department with right-sided pulsatile tinnitus, hearing loss, aural fullness, and disequilibrium. Twenty years earlier, she had presented to our facility with the same symptoms. She had had no other significant medical history at that time. Audiometry and an auditory brainstem response test had been performed, revealing a right-sided, asymmetric sensorineural hearing loss of 60 dB. Videonystagmography had revealed a 21% deficit on the right. MRI had revealed a heterogeneous, hyperintense lesion within the right petrous apex on both T1- and T2-weighted imaging, consistent with a cholesterol granuloma. A craniotomy with subtotal petrous apicectomy was performed via a right middle fossa approach for drainage of the cholesterol granuloma.

When the patient came to our office 20 years later, CT and MRI images revealed a right-sided lesion that was heterogeneously hyperintense on T 1 -weighted (figure 1, A) and isointense to hyperintense on T2-weighted MRI (figure 1, B), which is consistent with a paraganglioma. On CT, the lesion appeared as an opacified, expansile lesion (figure 2).

The patient elected to undergo stereotactic radiosurgery for treatment of this presumed paraganglioma. She had no complications from her treatment, with no resultant cranial nerve deficits, and the tumor size has been stable on follow-up.

Discussion

Cholesterol granuloma of the petrous apex became a recognized entity in the 1980s. (11-13) The exact etiology of cholesterol granuloma, however, remains controversial. Although it is widely accepted that the pathogenesis of cholesterol granuloma involves anaerobic catabolism of blood and blood products, the source of blood products within the temporal bone and clivus remains a topic of discussion.

Traditionally, blood products were thought to arise from transudative hemorrhage within air cells of the temporal bone secondary to negative pressure as a function of chronic eustachian tube dysfunction, also known as the "obstruction-vacuum theory." (14-19) However, Jackler and Cho challenge this assertion, stating that: (1) the negative pressure incited by eustachian tube dysfunction is not likely sufficient to cause hemorrhage; (2) cholesterol granuloma occurs only in well-pneumatized temporal bones, which are not present in patients with chronic eustachian tube dysfunction; and (3) once air cells are filled with fluid, the negative pressure should equalize and the process should discontinue. (19) Alternatively, they propose that the source of hemorrhage is dehiscence of the bony partition between the petrous apex air cell system and the bone marrow in the anterior temporal bone and clivus. (19)

This article describes a patient presenting with metachronous cholesterol granuloma and paraganglioma. Our case points to the possibility that paragangliomas might be associated with the formation of cholesterol granulomas of the petrous apex. We propose that microscopic bleeding from the paraganglioma was the source of blood and blood products responsible for the formation of the cholesterol granuloma in our patient.

In conclusion, the metachronous presentation of cholesterol granuloma in a patient with a paraganglioma provides a potential alternative source of hemorrhage in the pathogenesis of cholesterol granuloma in a select subgroup of patients.

References

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(16.) Rosenberg RA, Hammerschlag PE, Cohen NL, et al. Cholesteatoma vs. cholesterol granuloma of the petrous apex. Otolaryngol Head Neck Surg 1986;94(3):322-7.

(17.) Main TS, Shimada T, Lira DJ. Experimental cholesterol granuloma. Arch Otolaryngol 1970;91(4):356-9.

(18.) Nager GT, Vanderveen TS. Cholesterol granuloma involving the temporal bone. Ann Otol Rhinol Laryngol 1976;85(2 Pt 1):204-9.

(19.) Jackler RK, Cho M. A new theory to explain the genesis of petrous apex cholesterol granuloma. Otol Neurotol 2003;24(1):96-106.

Selena E. Heman-Ackah, MD, MBA; Tina C. Huang, MD

From the Department of Otolaryngology-Head and Neck Surgery, University of Minnesota, Minneapolis.

Corresponding author: Tina C. Huang, MD, MMC 396, 420 Delaware Ave., SE, Minneapolis, MN 55455. Email: huang081@umn.edu

Previous presentation: This article has been updated from its previous presentation as a poster at the Triological Society Combined Southern and Middle Section Meeting; January 8-11, 2009; Bonita Springs, Florida.
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Title Annotation:ORIGINAL ARTICLE
Author:Heman-Ackah, Selena E.; Huang, Tina C.
Publication:Ear, Nose and Throat Journal
Article Type:Report
Date:Sep 1, 2013
Words:1439
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