Gone but Not Forgotten

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Identifier walsh_2021_s2_c5
Title Gone but Not Forgotten
Creator Jonathan Micieli; Adriana Krizova; Walter Montanera
Affiliation (JM) (AK) (WM) University of Toronto, Toronto, Canada
Subject Optic Neuropathy; Malignant Melanoma
Description The persistent NLP vision and interval development of a CRAO raised concern for neoplastic optic nerve infiltration. Re-review of the MRI demonstrated T1-weighted hyperintensity in the left optic nerve, which is seen in melanoma and unusual of other neoplastic infiltrative/inflammatory lesions. The patient was specifically asked regarding a history of melanoma and recalled having a very small lesion removed from her upper back 10.5 years prior. The pathology reports from that time were retrieved and malignant melanoma without ulceration (T1a; 18 x 8 x 4mm; vertical height 0.68mm) was documented.[1] At that time, the only recommended treatment was a larger excision to ensure no residual melanocytic lesion was evident. The subsequent pathology report was clear and no further treatment was recommended given the negligible risk of recurrence or metastasis with a tiny lesion of vertical depth 0.68mm. With this new information, she underwent CT chest/abdomen/pelvis and this revealed numerous liver lesions with the largest measuring 1.1cm and a solitary 0.9cm pulmonary nodule. Biopsy of a liver lesion showed tumor cells positive for HMB-45 and S100, consistent with a diagnosis of melanoma. A final diagnosis of late recurrence (>10 years) of upper back cutaneous melanoma with metastases to the optic nerve, liver and lung was made. The patient was referred for radiation therapy to the left optic nerve (20Gy in 5 fractions). She was also referred to medical oncology and was enrolled in a clinical trial involving an investigational drug with ipilimumab and nivolumab. This treatment was complicated by autoimmune hepatitis and required treatment with intravenous and oral corticosteroids. Repeat MRI 4 months later showed interval improvement in the left optic nerve/optic nerve sheath enhancement and repeat CT abdomen 7 months later showed interval reduction in the size of the liver metastases. She remained NLP OS at 6 month follow-up.
History A 52 -year-old healthy woman presented with a 1-week history of blurred vision and 'soreness' in her left eye. Neuro-ophthalmic examination revealed a visual acuity of 20/20 OD, 20/40 OS, left RAPD and left superior arcuate defect on Humphrey visual field testing. Dilated fundus examination demonstrated a right 'disc-at-risk' and moderate left optic disc edema. She reported having 2 cats at home with a recent scratch on her leg and also had symptoms suggestive of obstructive sleep apnea (OSA). A differential diagnosis of neuroretinitis, non-arteritic anterior ischemic optic neuropathy (NAION) and optic neuritis was considered, but infectious workup was negative and polysomnography was not diagnostic of OSA. She was treated empirically with ciprofloxacin for presumed cat-scratch neuroretinitis, but her left eye vision significantly worsened to no light perception (NLP) 2 weeks after the initial visit. There was now severe left optic disc edema, mild venous dilation and tortuosity and significant intraretinal fluid in the macula. MRI brain and orbits with contrast revealed longitudinally extensive thickening and enhancement of the left optic nerve with surrounding fat stranding and no signs of demyelination in the brain. This was thought to represent severe optic neuritis, but AQP4-IgG, MOG-IgG and vasculitis workup were negative and chest X-ray was normal. She was treated with intravenous methylprednisolone 1gram daily for 5 days followed by Prednisone 1mg/kg daily, but her vision remained NLP 1 week later. She was referred for plasmapheresis and after the first session, she had worsening left eye pain. Repeat examination demonstrated a visual acuity of NLP OS and worsening left optic disc edema, diffuse retinal edema and a cherry-red -spot, indicating the interval development of a central retinal artery occlusion (CRAO). Repeat MRI brain/orbits with contrast showed decreased enhancement within the left optic nerve. A diagnostic test was performed.
Disease/Diagnosis Optic nerve infiltration from late recurrence of upper back cutaneous melanoma
Date 2021-02
References 1. Mohr P, Eggermont AMM, Hauschild A, Buzaid A. Staging of cutaneous melanoma. Ann Oncol. 2009;(Suppl 6):vi14-21. 2. D'souza NM, Nguyen HD, Smith SV, Nagarajan P, Diab A, Allen RC, Gombos DS, Lee AG. Metastatic melanoma of the optic nerve sheath. Neuro-Ophthalmology 2018;42:187-190.
Language eng
Format video/mp4
Type Image/MovingImage
Source 53rd Annual Frank Walsh Society Meeting
Relation is Part of NANOS Annual Meeting 2021
Collection Neuro-Ophthalmology Virtual Education Library: Walsh Session Annual Meeting Archives: https://novel.utah.edu/Walsh/
Publisher North American Neuro-Ophthalmology Society
Holding Institution Spencer S. Eccles Health Sciences Library, University of Utah
Rights Management Copyright 2021. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright
ARK ark:/87278/s690834c
Setname ehsl_novel_fbw
ID 1697345
Reference URL https://collections.lib.utah.edu/ark:/87278/s690834c