We present the case of a 54-year-old woman who presented with right facial weakness
and right-sided hearing loss and was found to have an erosive mass of the right jugular
bulb. On initial examination, otomicroscopy showed a pulsating violaceous mass in
the middle ear arising from the hypotympanum impinging on the tympanic membrane, and
the patient had a House-Brackman II facial palsy. The audiogram demonstrated a right
mild-to-moderate conductive hearing loss. The HRCT showed a right-sided irregularly
shaped mass of the middle ear and mastoid encasing the tympanic and mastoid segments
of the facial nerve, with moth-eaten erosion of the jugular plate. MRI T1 sequence
was hypointense, T2 was isointense and T1 postcontrast showed intense enhancement
of this mass. Overall, the patient’s presentation was highly concerning for jugular
paraganglioma. All management options were discussed with the patient. Urine metanephrines
testing was unremarkable. Genetic testing was also pursued and revealed no detectable
alterations to any of the 77 paraganglioma-associated genes.
Ultimately, the patient elected to pursue surgical resection. She was referred to
vascular neurosurgery for a preoperative same-day angiogram and embolization. On her
angiogram, no tumoral blush or feeding arteries were identified, so no embolization
was performed. Later that day she was taken for surgery, where a cortical mastoidectomy
was performed. At this point tumor was seen filling the mastoid, however, the lesion
was solid, tan-yellow, and well-circumscribed. This non-vascular lesion did not appear
grossly consistent with paraganglioma and appeared more like schwannoma. When the
nerve stimulator probe (at 0.1mA. contacted the tumor, all branches of the facial
nerve activated. A piece of tumoral tissue was biopsied and sent for frozen section.
This showed pathologic features including nuclear palisading (Verocay bodies), which
was indeed consistent with facial nerve schwannoma. Given these findings, the decision
was made to terminate the procedure to preserve facial function. The patient was seen
in follow-up and found to be healing well with stable facial function. A joint plan
was made to proceed with close observation and re-consider surgical resection should
her facial function worsen beyond a House Brackmann 3/6. This patient represents a
diagnostic dilemma–diagnostic considerations and different treatment algorithms will
be presented.
