Primary Paraganglioma of the Facial Canal: An Evidence‐Based Approach

Abstract

Objectives To perform an evidence-based review evaluating presenting symptoms, imaging, and management for primary paragangliomas of the facial canal (PPFCs). Data Sources PubMed/MEDLINE, Embase, Cochrane Library, and Web of Science. Review Methods Studies were assessed for quality of evidence and bias with the Cochrane bias tool, GRADE, and MINORS criteria. Demographic data, imaging modalities, management strategies, and status at last follow-up were obtained. Results Sixteen studies met inclusion criteria. In total, 21 patients with PPFCs were identified, 19 of which were histologically confirmed. Most common presenting symptoms included unilateral facial nerve dysfunction (n = 14, 73.7%) and pulsatile tinnitus (n = 8, 42.1%). Mean time from reported onset of facial dysfunction was 17.8 months. Computed tomography findings included an expanded descending facial nerve canal (n = 13, 76.5%). All cases with magnetic resonance imaging reported enhancement with contrast. Of the 18 patients who had surgery, 16 (88.9%) underwent full tumor resection while 1 (5.6%) had partial tumor debulking with adjuvant radiotherapy. Overall improvement in facial weakness was documented in 5 of 9 patients (55.6%) with initial facial nerve dysfunction and >6-month follow-up. No evidence of tumor recurrence was reported. Conclusions PPFCs are extraordinarily rare vascular neoplasms of the temporal bone. Early imaging with both computed tomography and magnetic resonance imaging is essential for narrowing the differential diagnosis, assessing the extent of tumor invasion, and accurate surgical planning. Surgical tumor resection with subsequent facial nerve reconstruction is recommended for patients with facial nerve dysfunction, while tumor biopsy or debulking may be indicated when normal facial movement in present.

Primary Middle Ear Meningioma with Intact Tympanic Membrane: A Case Report and Literature Review

“…Another unique feature of the MRI is the “dural tail sign.” The dural tail sign is an en plaque linear dural enhancement along the floor of the middle or posterior cranial fossa. 18 Glomus jugulare paraganglioma 19 can be distinguished from it according to the characteristics of the “salt-pepper sign” or “serpentine effusion” on MRI, and enhanced MRI has higher sensitivity. 20 Gadolinium-enhanced MRI is superior to CT scan in distinguishing peripheral extension of intracranial meningioma and primary extracranial meningioma.…”

Facial Nerve Paraganglioma Masquerading as Bell palsy: A Reconstructive Pitfall

“…The clinical presentation and treatment methods described in the current case are thus exceptional. Yuhan et al 12 2020 recently published a systematic review of primary paraganglioma of the facial canal documents 21 reported cases in the literature found in case reports and case series. In their review the most common presenting symptom was facial nerve dysfunction, the most common CT finding was an expanded descending facial nerve canal, all studies with MRI findings noted enhancement with contrast.…”

“…11 A recent systematic review of primary paraganglioma of the facial canal documents 21 reported cases in the literature found in case reports and case series. 12 In this report, we describe the case of a 66-year old Hispanic female with ''BP'' that presented to the plastic surgery clinic with a complaint of left facial paralysis. After a thorough workup, a lesion involving the facial nerve from the tympanic segment to the stylomastoid foramen was discovered, ultimately the lesion was found to be a facial nerve paraganglioma…”

“…Much less is known about the exceptionally rare facial nerve paragangliomas – slow-growing benign neoplasms arising from foci of neural crest-derived chemoreceptor tissue 11 . A recent systematic review of primary paraganglioma of the facial canal documents 21 reported cases in the literature found in case reports and case series 12 …”