• Users Online: 453
  • Print this page
  • Email this page

Table of Contents
Year : 2021  |  Volume : 3  |  Issue : 1  |  Page : 37

Optic nerve sheath carcinomatosis masquerading as optic perineuritis: The first sign of gastric cancer relapse

1 Department of Ophthalmology, Hospital Prof. Doutor Fernando Fonseca E.P.E, Amadora, Lisbon, Portugal
2 Immunomediated Systemic Diseases Unit, Hospital Prof. Doutor Fernando Fonseca E.P.E., Amadora, Lisbon, Portugal

Date of Submission17-Oct-2021
Date of Decision26-Oct-2021
Date of Acceptance26-Oct-2021
Date of Web Publication22-Nov-2021

Correspondence Address:
Dr. Joana Roque
Department of Ophthalmology, Hospital Prof. Doutor Fernando Fonseca, IC19, 2720- 276 Amadora, Lisbon
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/pajo.pajo_113_21

Rights and Permissions

Optic perineuritis (OPN) is a rare inflammatory disease involving the optic nerve sheath. Most cases are idiopathic, but associations with a variety of infectious and inflammatory conditions have been described. Patients with suspected OPN should also be investigated for neoplasms in the form of optic nerve sheath meningioma or meningeal carcinomatosis, given they share important clinical and radiological features. We present a case of suspected idiopathic bilateral OPN that was particularly challenging, eventually revealing a neoplastic etiology.

Keywords: Meningeal carcinomatosis, optic nerve sheath metastasis, optic perineuritis

How to cite this article:
Roque J, Silva D, Grima B, Prieto I. Optic nerve sheath carcinomatosis masquerading as optic perineuritis: The first sign of gastric cancer relapse. Pan Am J Ophthalmol 2021;3:37

How to cite this URL:
Roque J, Silva D, Grima B, Prieto I. Optic nerve sheath carcinomatosis masquerading as optic perineuritis: The first sign of gastric cancer relapse. Pan Am J Ophthalmol [serial online] 2021 [cited 2023 Jan 27];3:37. Available from: https://www.thepajo.org/text.asp?2021/3/1/37/330935

  Introduction Top

Optic perineuritis (OPN) is a rare inflammatory disease involving the optic nerve sheath. Most cases are idiopathic, but OPN has also been associated with a variety of infectious and inflammatory conditions, including syphilis,[1] tuberculosis,[2] sarcoidosis,[3] giant cell arteritis,[4] and polyangiitis with granulomatosis.[5] Typical presentation includes unilateral vision loss characteristically progressing over weeks. Besides an extensive search for secondary causes, the diagnostic workup of suspected OPN always includes neuroradiological studies. Magnetic resonance imaging (MRI) typically shows optic nerve sheath enhancement, while the optic nerve itself is usually normal.

Furthermore, when approaching a patient with suspected OPN, some differential diagnoses must be considered, including optic neuritis, orbital inflammatory syndrome, and especially neoplasms in the form of optic nerve sheath meningioma or meningeal carcinomatosis. We present a case of suspected bilateral OPN that was particularly challenging, eventually revealing a neoplastic etiology.

  Case Report Top

We report the case of a 68-year-old male smoker with a history of gastric adenocarcinoma (T4N3M0) treated with total gastrectomy and adjuvant and neoadjuvant chemotherapy 2 years earlier. He was referred to the ophthalmology department for a 1-month history of progressive vision loss in the left eye (OS).

On examination, the patient presented a relative afferent pupillary defect OS. Best-corrected visual acuity was 20/20 in the right eye (OD) and hand movements OS.

Fundus examination showed bilateral disc edema with peripapillary hemorrhages [Figure 1]. OS also presented macular edema, associated with pre-macular and slight vitreous hemorrhages. Optical coherence tomography (OCT) scans confirmed these findings [Figure 2]. The remainder of ophthalmological, neurological, and systemic examination was unremarkable.
Figure 1: Fundus photos on presentation showing bilateral generalized optic disc edema with peripapillary hemorrhages most prominent in the left eye

Click here to view
Figure 2: (a and b) Circular peripapillary Optical coherence tomography scans of both eyes showing significant edema of retinal nerve fiber layer. (c) Optical coherence tomography B-scan centered in the fovea OS showing significant disc edema associated with subretinal fluid and macular edema

Click here to view

Urgent computed tomography (CT) of the head and orbits, along with CT cerebral venography, revealed only a discrete supratentorial hydrocephaly. A lumbar puncture was subsequently performed to exclude raised intracranial pressure and central nervous system infection or malignancy. Opening pressure was not elevated and cytochemical cerebrospinal fluid (CSF) examination was unremarkable.

An MRI of the brain and orbits carried out 2 weeks later revealed an abnormal enhancement surrounding the intraorbital portion of the optic nerves [Figure 3], suspected of bilateral OPN. This perineural contrast enhancement presented the characteristic “tram track” and “doughnut” signs on axial and coronal scans, respectively. On the left, there was also some enhancement of adjacent tissues.
Figure 3: T2-weighted Magnetic resonance imaging with gadolinium contrast. (a) Axial section showing “tram track” enhancement (➤) of both optic nerve sheaths. (b) Bilateral “doughnut-shaped” enhancement (➜) in a coronal section

Click here to view

The patient underwent an extensive workup for autoimmune conditions (sarcoidosis, giant-cell arteritis, granulomatous polyangiitis, neuromyelitis optica, and anti-MOG disease) as well as infectious diseases (syphilis, tuberculosis, Lyme disease, and cat-scratch disease). All results were negative. Moreover, thoracoabdominal and pelvic CT scans showed no evidence of relapsed gastric carcinoma.

The diagnosis of idiopathic bilateral OPN was assumed, and he was given a high-dose course of corticosteroids, followed by a slow-tapering regimen. Within 3 weeks, clinical improvement was only partial, with no vision recovery. In addition, the patient had to be hospitalized again because of new neurological symptoms (intense headaches, dizziness, and inferior limb paralysis).

Meningeal carcinomatosis was again considered in the differential diagnosis. A second lumbar puncture was performed and revealed an increased intracranial pressure as well as the presence of neoplastic cells. These findings confirmed the metastatic recurrence of gastric carcinoma, so intrathecal chemotherapy sessions with methotrexate were initiated. Despite treatment, the clinical course continued to deteriorate, culminating in the patient's death 3 months after initial presentation.

  Discussion Top

This report illustrates a case of bilateral optic nerve sheath metastasis as the sole manifestation of relapsed gastric carcinoma. Meningeal carcinomatosis is a rare and usually terminal complication of malignant neoplasms, most commonly adenocarcinomas.[6] It results from cancer cells spreading to the leptomeninges. Despite treatment with radiation or intrathecal chemotherapy, expected median survival of patients with meningeal carcinomatosis is only 2–6 months.[6]

The optic nerves are often affected, causing sheathing or even infiltration,[7] so it is mandatory as a differential diagnosis in cases of optic neuritis and perineuritis. Clinically, these conditions have some distinctive features. Demyelinating optic neuritis typically affects young females, with acute monocular vision loss associated with painful eye movements. OPN typically affects an older age group. The clinical presentation may mimic optic neuritis, but the vision loss appears to be more progressive, over several weeks, and more often shows sparing of central vision.[8] Treatment of idiopathic OPN with high-dose corticosteroids leads to prompt vision recovery; however, prolonged therapy and slow tapering are essential to minimize the risk of recurrence.[9]

Meningeal carcinomatosis usually occurs in patients with widely disseminated and progressive systemic cancer (>70%), but it can present after a disease-free interval (20%) and even be the first manifestation of cancer.[10] Clinical manifestations of meningeal carcinomatosis are highly variable and depend on the affected structures, both in central and peripheral nervous systems. Although ocular involvement represents a frequent manifestation, isolated optic nerve sheath infiltration is not a common finding, as reported by Lanfranconi et al.[7]

Radiologically, optic nerve sheath carcinomatosis shares characteristic features with OPN, as well as optic nerve sheath meningioma. MRI scans in these conditions typically demonstrate contrast enhancement around the intraorbital portion of the optic nerve. The “tram track” sign and “doughnut-shaped” perineural enhancement, as seen in our patient, can be present in any of these optic nerve sheath conditions.

When suspecting meningeal carcinomatosis, CSF cytology is the gold standard. However, the first examination is often negative for malignant cells.[10] This was a confounding factor in our patient since initially, he presented clinical and radiographic features of OPN.

Clinicians should be aware that idiopathic perineuritis is considered a diagnosis of exclusion and an inadequate response to corticosteroid treatment should prompt further investigation.

  Conclusion Top

This case highlights the importance of differentiating between OPN and neoplastic involvement of the optic nerve sheath such as metastatic disease. MRI may show similar findings, but CSF analysis is mandatory to differentiate the diseases. In patients with cancer history, a high level of clinical suspicion is crucial to diagnose meningeal carcinomatosis in the absence of any other systemic findings.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Toshniwal P. Optic perineuritis with secondary syphilis. J Clin Neuroophthalmol 1987;7:6-10.  Back to cited text no. 1
Yates WB, Nothling S, Lawlor M. Optic perineuritis due to tuberculosis. J Neuroophthalmol 2019;39:257-9.  Back to cited text no. 2
Yu-Wai-Man P, Crompton DE, Graham JY, Black FM, Dayan MR. Optic perineuritis as a rare initial presentation of sarcoidosis. Clin Exp Ophthalmol 2007;35:682-4.  Back to cited text no. 3
Morotti A, Liberini P, Padovani A. Bilateral optic perineuritis as the presenting feature of giant cell arteritis. BMJ Case Rep 2013;2013:bcr2011007959.  Back to cited text no. 4
Takazawa T, Ikeda K, Nagaoka T, Hirayama T, Yamamoto T, Yanagihashi M, et al. Wegener granulomatosis-associated optic perineuritis. Orbit 2014;33:13-6.  Back to cited text no. 5
Chamberlain MC. Neoplastic meningitis. Oncologist 2008;13:967-77.  Back to cited text no. 6
Lanfranconi S, Basilico P, Trezzi I, Borellini L, Franco G, Civelli V, et al. Optic neuritis as isolated manifestation of leptomeningeal carcinomatosis: A case report and systematic review of ocular manifestations of neoplastic meningitis. Neurol Res Int 2013;2013:892523.  Back to cited text no. 7
Purvin V, Kawasaki A, Jacobson DM. Optic perineuritis: Clinical and radiographic features. Arch Ophthalmol 2001;119:1299-306.  Back to cited text no. 8
Tatsugawa M, Noma H, Mimura T, Funatsu H. High-dose steroid therapy for idiopathic optic perineuritis: A case series. J Med Case Rep 2010;4:404.  Back to cited text no. 9
Djukic M, Trimmel R, Nagel I, Spreer A, Lange P, Stadelmann C, et al. Cerebrospinal fluid abnormalities in meningeosis neoplastica: A retrospective 12-year analysis. Fluids Barriers CNS 2017;14:7.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]


    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

  In this article
Case Report
Article Figures

 Article Access Statistics
    PDF Downloaded96    
    Comments [Add]    

Recommend this journal