Identifier |
walsh_2019_s3_c4 |
Title |
At the Crossroads |
Creator |
Konstantinos Douglas, MD, DVM, MBA |
Subject |
Intracranial Tumors; Cranial Nerve Palsies; Facial Pain; Perineural Invasion |
History |
A 54-year-old female former smoker presented with vertical diplopia, left facial pain and left forehead weakness. She previously had thyroidectomy for a noncancerous nodule and abdominal hysterectomy for fibroids. She reported a two-year history of worsening facial pain and numbness that started as a sore spot above the left eyebrow, and over months progressed to involve the left forehead and cheek. This pain was initially responsive to steroids but became persistent, severe, and unresponsive to treatment. Within a few months of the onset of the pain, the patient developed oblique binocular diplopia that also worsened over time. In addition, she lost the ability to move the forehead on that side, although the rest of the face appeared normal. Exam was notable for left-sided cranial nerve findings: partial CNIII palsy, complete CNIV and CNVI palsies, left-sided hypesthesia in the V1-2 distribution, and weakness of the temporal branch of left frontal nerve. Later, left eye chemosis, injection and inferior keratopathy were also present. Skin and ENT exams were normal. MRI with contrast revealed asymmetric enhancement of left cavernous sinus; on further review there was extension along the left frontal and vidian nerves. CT chest/abdomen/pelvis showed a benign hepatic cyst. PET scan only showed reduced uptake in the extraocular muscles, secondary to the cranial nerve palsies. An extensive laboratory work-up was unrevealing, including serologies for connective tissue diseases, ANCA, sarcoidosis, Lyme and syphilis. Four lumbar punctures yielded normal CSF and cell counts, including cytology and flow. The patient and was managed with escalating doses of steroids, which were initially given orally but then transitioned to IV. She was admitted to an outside hospital for severe refractory pain, and transferred to our institution. A diagnostic left frontal nerve biopsy was obtained, and showed moderately differentiated, keratinizing squamous cell carcinoma with intraneural and perineural involvement. |
Disease/Diagnosis |
Intracranial squamous cell carcinoma with unknown primary origin, which in addition to involvement of the cranial nerves in the cavernous sinus (III, IV, VI, V1, V2) included branches of the ipsilateral facial nerve. |
Date |
2019-03 |
References |
[1] Pierre R Bourque and others, ‘Combined Isolated Trigeminal and Facial Neuropathies from Perineural Invasion by Squamous Cell Carcinoma: A Case Series and Review of the Literature', Journal of Clinical Neuroscience, 2017 <http://dx.doi.org/https://doi.org/10.1016/j.jocn.2016.09.022>. [2] Serkan Erkan and others, ‘Clinical Perineural Invasion of the Trigeminal and Facial Nerves in Cutaneous Head and Neck Squamous Cell Carcinoma: Outcomes and Prognostic Implications of Multimodality and Salvage Treatment', Head and Neck, 2017 <http://dx.doi.org/10.1002/hed.24607>. |
Language |
eng |
Format |
video/mp4 |
Type |
Image/Moving |
Source |
2019 North American Neuro-Ophthalmology Society Annual Meeting |
Relation is Part of |
NANOS Annual Meeting 2019: Frank B. Walsh Session 3 |
Collection |
Neuro-Ophthalmology Virtual Education Library - Walsh Session Annual Meeting Archives https://novel.utah.edu/Walsh/index3.html |
Publisher |
Spencer S. Eccles Health Sciences Library, University of Utah |
Holding Institution |
Spencer S. Eccles Health Sciences Library, University of Utah, 10 N 1900 E SLC, UT 84112-6030 |
Rights Management |
Copyright 2019. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
ARK |
ark:/87278/s6m94ssj |
Setname |
ehsl_novel_fbw |
ID |
1431971 |
Reference URL |
https://collections.lib.utah.edu/ark:/87278/s6m94ssj |