Abstract
Purpose
- 1.
Describe the presentation, imaging, and outcome of two cases of paraganglioma of the facial canal at our institution.
- 2.
Summarize existing literature to better understand this lesion.
- 3.
Clarify terminology.
Methods
Retrospective chart review at single tertiary academic referral center. Literature review using the PubMed electronic database.
Results
There are 12 cases of histologically-proven paraganglioma of the facial canal published in the English literature. We present two additional cases that were encountered at our institution. We also include three additional cases from a separate institution that have been accepted for publication in a different journal. We found that patients most commonly present with slowly progressive facial paralysis; though paralysis can be acute. The second most common symptom was pulsatile tinnitus, which was the only symptom in one patient. Radiographically, tumor location was in the descending segment in all but one case. The mass was often centered directly over the stylomastoid foramen, mimicking a parotid mass. We found circumferential widening of the proximal fallopian canal, and a “moth-eaten” bony destruction distally on CT imaging in several patients. In patients with poor facial nerve function (HB IV-VI), complete tumor removal with nerve sacrifice followed by great auricular or sural nerve grafting was performed with acceptable facial function results.
Conclusions
Paraganglioma of the facial canal remains a rare etiology of progressive facial paralysis. The lesions are found in the distal descending segment, or centered over the stylomastoid foramen 94% of the time. In patients with poor facial function, surgical removal with nerve sacrifice, followed by great auricular or sural nerve grafting, yields an acceptable functional result.
1
Introduction
In the patient presenting with progressive facial paresis, the differential diagnosis remains fairly broad. However, when that patient also has an imaging study that reveals a mass lesion along the course of the facial nerve, particularly the descending portion, that differential becomes more focused. Of the options, paraganglioma is the least common and the most newly recognized. There appears to be a lack of consensus on what these lesions should be defined as. Indeed, to date there are only 12 reports for a total of 14 patients published in the English literature. Analysis of this body of work is made more difficult when three different names (paraganglioma of the facial nerve, paraganglioma of the facial canal, and glomus faciale) are used to describe the lesion.
Herein, we add our experience with two patients, and summarize the presentation and imaging of existing reports as well as those of three patients in a separate pending publication from another institution. In doing so, it is our hope to better define this relatively unrecognized pathology, discuss its presentation, exam findings, and imaging characteristics. We also hope to move toward a unified diagnostic term for this lesion. Armed with increased knowledge of this tumor’s presentation, perhaps clinicians may change their pre-operative discussions and operative plan in patients presenting with this lesion.
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