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Year : 2021  |  Volume : 1  |  Issue : 4  |  Page : 782-784

Isolated intracranial Rosai–Dorfman disease presenting as compressive optic neuropathy

Department of Pediatric Ophthalmology and Strabismus, Sankara Eye Hospital, Bengaluru, Karnataka, India

Date of Submission16-Mar-2021
Date of Acceptance10-May-2021
Date of Web Publication09-Oct-2021

Correspondence Address:
Dr. Sowmya Raveendra Murthy
Department of Pediatric Ophthalmology and Strabismus, Sankara Eye Hospital, Varthur Main Road, Kundlahalli Gate, Bengaluru - 560 037, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_559_21

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A 56-year-old female presented with bilateral progressive painless loss of vision. Examination showed a relative afferent pupillary defect in the left eye with temporal disc pallor and visual field loss. MRI (magnetic resonance imaging) brain revealed a suprasellar mass for which mass excision via frontotemporal craniotomy was done. Histopathology examination of the mass revealed foamy histiocytes with emperipolesis, S-100 marker was positive. Thus, a diagnosis of Rosai–Dorfman disease (RDD) was made. Our case describes a rarely found isolated intracranial RDD presenting as compressive optic neuropathy and vision loss in an elderly female.

Keywords: Isolated Intracranial mass, Rosai–Dorfman disease

How to cite this article:
Patil A, Raveendra Murthy S, Mohamed A. Isolated intracranial Rosai–Dorfman disease presenting as compressive optic neuropathy. Indian J Ophthalmol Case Rep 2021;1:782-4

How to cite this URL:
Patil A, Raveendra Murthy S, Mohamed A. Isolated intracranial Rosai–Dorfman disease presenting as compressive optic neuropathy. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Oct 26];1:782-4. Available from: https://www.ijoreports.in/text.asp?2021/1/4/782/327696

Rosai–Dorfman disease (RDD) is a non-Langerhans cell histiocytosis characterized by the accumulation of activated histiocytes in lymph node sinuses and extranodal sites.[1],[2] Classically seen in children and young adults, this presents as a massive bilateral cervical lymphadenopathy with fever, leukocytosis, and raised erythrocyte sedimentation rate.[3] About 43% of patients have extranodal manifestations that include eye, upper respiratory tract, skin, salivary glands, bone, meninges, central nervous system, and testis.[1] The ophthalmic manifestations occur in 11% of cases and include involvement of orbital soft tissue, eyelids, lacrimal gland, conjunctiva, and uvea.[2]

Approximately 70 cases of isolated intracranial involvement without nodal involvement are described.[4] There are two previous case reports of RDD presenting as a compressive optic neuropathy in the elderly age-group.[3],[4],[5],[6],[7] We report a case of isolated intracranial RDD presenting as compressive optic neuropathy in an elderly female.

  Case Report Top

A 56-year-old female presented to the hospital with complaints of painless progressive loss of vision in both eyes. The patient had a previous history of chronic rhinosinusitis and had undergone FESS (functional endoscopic sinus surgery) surgery. There was no history of any other systemic illness.

The BCVA (best-corrected visual acuity) was right eye 6/9, N6, and left eye 6/9, N6. Bilateral color vision was defective on Ishihara charts. Contrast sensitivity tested by Pelli–Robson test was 1.5 log units in both eyes.

The anterior segment evaluation revealed quiet pseudophakia with a relative afferent pupillary defect in the left eye. Fundus evaluation showed bilateral temporal disc pallor [Figure 1]a and [Figure 1]b.
Figure 1: (a) Right eye showing temporal disc pallor. (b) Left eye showing temporal disc pallor

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Humphrey field analysis 30-2 showed a generalized depression of field in the right eye and inferonasal sparing of the field in the left eye [Figure 2]a and [Figure 2]b. The 120-point screening showed an inferior altitudinal field defect in the right eye with few superior peripheral depressed points, whereas the left eye visual field showed that only inferonasal field was spared. Optic nerve head optical coherence tomography was done, which revealed retinal nerve fiber layer (RNFL) thinning; right eye RNFL thickness 56 μm, left eye RNFL thickness 54 μm.
Figure 2: (a) Right eye showing generalized depressed fields. (b) Left eye showing inferonasal sparing of field

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In view of an afferent pupillary defect, temporal disc pallor, and visual field changes, an MRI brain was ordered, which showed a suprasellar intensely enhancing mass suggestive of a suprasellar meningioma [Figure 3]a and [Figure 3]b. A diagnosis of compressive optic neuropathy was made, and the patient was referred for neurosurgeon's opinion.
Figure 3: (a) MRI brain coronal section showing enhancing suprasellar mass (blue arrow). (b) MRI brain sagittal section showing enhancing suprasellar mass (blue arrow)

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The patient underwent a frontotemporal craniotomy for suprasellar mass excision, and the excised mass was sent for histopathological evaluation. The histopathological evaluation showed lymphoplasmacyte-rich histiocytic lesion with secondary stromal and meningeal changes [Figure 4]a. Foamy histiocytes showing emperipolesis were detected, and S-100 marker was positive on immunohistochemistry [Figure 4]b. A diagnosis of intracranial RDD was established.
Figure 4: (a) Hematoxylin and eosin staining showing lesion with lymphohistiocytic infiltrate and plasma cells, emperipolesis (blue arrow). (b) S-100 marker positive lesion

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The patient was started on systemic tapering steroids for the residual disease but showed no response. She was further treated with external radiotherapy 29Gy/10 fractions.

  Discussion Top

RDD or sinus histiocytosis with massive lymphadenopathy is characterized by proliferation and accumulation of non-Langerhans cell histiocytes in the lymph node sinuses and extranodal sites.[1]

The disease usually presents with massive painless bilateral cervical lymphadenopathy, fever, weight loss, and night sweats, and is seen in children and young adults.[1],[6] The extranodal sites involved in the decreasing order of frequency are skin, nasal cavity, paranasal sinuses, eyelid, orbit, bone, and central nervous system.[1] The reported ophthalmic manifestations of RDD include lymphoproliferation in the soft tissue of orbit, eyelid, lacrimal gland, uvea, and compressive optic neuropathy.[1],[3]

Extranodal RDD presents rarely with isolated progressive visual loss due to compressive optic neuropathy, and only two previous cases have been reported in elder patients.[3],[7] Compressive optic neuropathy in extranodal RDD could occur secondary to an orbital mass or as an intracranial RDD involving the sella. The cause of compressive optic neuropathy was a suprasellar mass, and the patient presented with painless progressive visual loss in our case like the previously described cases. The vision loss was severe with a drop in vision to light perception in one of these cases as against our case. Visual field loss was seen in both eyes in our case unlike in one of the previous cases where one eye was unaffected.

The disease was not limited to the central nervous system in the earlier cases and caused additional manifestations of eyelid mass and epistaxis as against our case, where an isolated suprasellar mass was causing a compressive optic neuropathy.

Being primarily a meningeal process, the RDD may mimic a meningioma on radiodiagnosis[7] as was in our case as well as one of the previously described cases. Neuroimaging may also reveal simultaneous involvement of paranasal sinuses in the disease process, which was not seen in our case.[3]

Hematoxylin and eosin staining showing emperipolesis, which means an intact cell within the cytoplasm of another cell and immunohistochemistry showing S-100, CD68 positive,[6] which is diagnostic for RDD was present in all cases.

RDD is usually self-limited and with spontaneous remission reported in up to 50% of cases.[6] Intervention is indicated in symptomatic cases involving critical locations. Surgical excision, radiotherapy, chemotherapy, and systemic steroids are the available modalities of treatment.[8] Since recurrences can occur, surveillance is indicated for relapse for at least 5 years.[9] Multimodality approach of treatment was followed in our case as well as in the previous cases.

  Conclusion Top

Our case is unique in presenting as mild visual disturbance and field loss with only temporal pallor of optic discs in isolated intracranial RDD. This highlights the importance of imaging and tissue biopsy in such cases.

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.


The histopathology Images are courtesy Dr. Nandeesh BN, Department of Neuropathology, National Institute of Mental Health and Neurosciences, Bengaluru.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Vemuganti GK, Naik MN, Honavar SG. Rosai dorfman disease of the orbit. J Hematol Oncol 2008;1:7.  Back to cited text no. 1
Abla O, Jacobsen E, Picarsic J, Krenova Z, Jaffe R, Emile JF, et al. Consensus recommendations for the diagnosis and clinical management of Rosai-Dorfman-Destombes disease. Blood 2018;131:2877-90.  Back to cited text no. 2
Shukla E, Nicholson A, Agrawal A, Rathod D. Extra nodal Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) presenting as asymmetric bilateral optic atrophy: An atypical ocular presentation. Head Neck Pathol 2016;10:414-7.  Back to cited text no. 3
Taufiq M, Khair A, Begum F, Akhter S, Shamim Farooq M, Kamal M. Isolated intracranial Rosai-Dorfman disease. Case Rep Neurol Med 2016;2016:1972594. doi: 10.1155/2016/1972594.  Back to cited text no. 4
McPherson CM, Brown J, Kim AW, DeMonte F. Regression of intracranial rosai-dorfman disease following corticosteroid therapy. Case Rep J Neurosurg 2006;104:840-4.  Back to cited text no. 5
Bruce-Brand C, Schneider JW, Schubert P. Rosai-Dorfman disease: An overview. J Clin Pathol 2020;73:697-705.  Back to cited text no. 6
Siu RC, Tan IL, Davidson AS, Robertson A, Fraser CL. Clinical reasoning: Compressive optic neuropathy secondary to intracranial Rosai-Dorfman disease. Neurology 2015;85:e89-92.  Back to cited text no. 7
Goyal G, Ravindran A, Young JR, Shah MV, Bennani NN, Patnaik MM, et al. Clinicopathological features, treatment approaches, and outcomes in Rosai-Dorfman disease. Haematologica 2020;105:348-57.  Back to cited text no. 8
Petzold A, Thom M, Powell M, Plant GT. Relapsing intracranial Rosai-Dorfman disease. J Neurol Neurosurg Psychiatry 2001;71:538-41.  Back to cited text no. 9


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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