A rare case of primary lymphoma of the ciliary body – A case report

Mahesh Shanmugam Palanivelu; Shwetha Suryakanth; Rajesh Ramanjulu; Divyansh Kailashchandra Mishra; Surbhi Agrawal

doi:10.4103/IJO.IJO_2934_22

CASE REPORT

Year : 2023 | Volume : 3 | Issue : 2 | Page : 377-379

A rare case of primary lymphoma of the ciliary body – A case report

Mahesh Shanmugam Palanivelu, Shwetha Suryakanth, Rajesh Ramanjulu, Divyansh Kailashchandra Mishra, Surbhi Agrawal
Department of Vitreoretina and Ocular Oncology, Sankara Eye Hospital, Bangalore, Bangalore, Karnataka, India

Date of Submission	03-Nov-2022
Date of Acceptance	04-Jan-2023
Date of Web Publication	28-Apr-2023

Correspondence Address:
Shwetha Suryakanth
Department of Vitreoretina and Ocular Oncology, Sankara Eye Hospital, Kundalahalli Gate, Varthur Main Road, Bangalore - 560 037, Karnataka
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJO.IJO_2934_22

Abstract

A 73-year-old lady presented with complicated cataract, white tumor hypopyon, and an elevated, nonpigmented mass at the root of iris. Magnetic resonance imaging (MRI) revealed ciliary body involvement suspicious of atypical melanoma. Patient underwent enucleation. Histopathology with immunohistochemistry (IHC) markers clinched the diagnosis of diffuse large B-cell lymphoma (DLBCL) of ciliary body, with positive lymphoma markers CD20, CD3, BCL2, and PAX5 and negative melanoma markers HMB45 and S100. Primary lymphoma of ciliary body, especially the DLBCL type, is rare. Our report shows that primary lymphoma of ciliary body may be considered as a differential in cases with atypical features. Histopathology and immunohistochemistry are valuable tools in diagnosing challenging cases.

Keywords: Ciliary body lymphoma, ciliary body tumors, histopathology, immunohistochemistry, uveal tumors

How to cite this article:
Palanivelu MS, Suryakanth S, Ramanjulu R, Mishra DK, Agrawal S. A rare case of primary lymphoma of the ciliary body – A case report. Indian J Ophthalmol Case Rep 2023;3:377-9

How to cite this URL:
Palanivelu MS, Suryakanth S, Ramanjulu R, Mishra DK, Agrawal S. A rare case of primary lymphoma of the ciliary body – A case report. Indian J Ophthalmol Case Rep [serial online] 2023 [cited 2023 Jun 2];3:377-9. Available from: https://www.ijoreports.in/text.asp?2023/3/2/377/375003

Tumors originating from the ciliary body can pose diagnostic challenges to the clinician owing to their anterior location, late presentation, and possibility of a multitude of differential diagnoses.^[1] Most patients with ciliary body tumors present with nonspecific ocular symptoms that may delay the diagnosis. Most studies report primary tumors of ciliary body to be rare, and the most common types are ciliary body melanoma followed by melanocytoma.^[2] We report a rare case of a biopsy-proven primary ciliary body lymphoma, which was initially suspected to be atypical melanoma of the ciliary body. There are only a few cases of primary ciliary body lymphoma reported in literature.^[3],[4],[5] Informed consent has been obtained from the patient to publish the case and the images, and it has been ensured that none of the images reveal patient's identity in any manner.

Case Report

A 73-year-old lady was referred to us for a suspected atypical choroidal melanoma. She had a history of sudden-onset diminution of vision in the right eye (RE) 4 months ago that was associated with pain and redness with no systemic illness. She was treated with topical steroids elsewhere with a working diagnosis of acute iridocyclitis. In view of poor response to topical therapy, she had also received a posterior sub-Tenon injection of triamcinolone acetonide. The pain and redness reduced; however, the vision continued to worsen over the next 3 months.

She consulted in another center, where she was further evaluated and was found to have a complicated cataract. B-scan ultrasonography showed a homogenous, hyperechoic lesion in the superonasal quadrant with retinal detachment. However, in view of the anterior location of the mass lesion, the entire lesion could not be captured on the B-scan. So, the size of the lesion could not be determined. Magnetic resonance imaging (MRI) of brain and orbits showed a mass lesion involving ciliary body and choroid, hypointense on T1-weighted images [Figure 1]a and iso- to hyperintense on T2-weighted images [Figure 1]b with intense homogenous post-contrast enhancement [Figure 1]c. The features were suggestive of amelanotic uveal melanoma since there was no hyperintensity on T1-weighted images that a typical melanoma with melanin pigment would exhibit. A peripheral smear examination showed leukocytosis with neutrophilia, with no abnormal cells. Ultrasonography of the abdomen showed no focus of malignancy. A provisional diagnosis of atypical choroidal melanoma of the RE was made, and the patient was referred to us for further management.

Figure 1: MRI of brain and orbits showing a mass lesion involving ciliary body and choroid, which was (a) hypointense on T1-weighted images and (b) iso- to hyperintense on T2-weighted images with (c) intense homogenous post-contrast enhancement. MRI = magnetic resonance imaging

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At presentation to us, her best corrected visual acuity was positive perception of light with inaccurate projection of rays in the affected eye and 6/6, N6 in the fellow eye. Slit-lamp examination of RE revealed circumcorneal congestion but no evidence of sentinel vessels. There was white tumor hypopyon of about 1 mm height consisting of large, white cells settled inferiorly in the anterior chamber. An elevated, nonpigmented mass was noted nasally at the root of iris [Figure 2]. There was dense complicated cataract obscuring the view of fundus. Her left eye (LE) was unremarkable. In view of few atypical features like absence of sentinel vessels, nonpigmented mass lesion, and presence of hypopyon, the diagnosis of melanoma was unconvincing. Hence, we decided to evaluate further.

Figure 2: Slit-lamp photograph of the right eye showing complicated cataract, white tumor hypopyon, and elevated, nonpigmented mass at the root of iris

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Anterior chamber tap was done and sent for cytological examination. Cytology was positive for lymphocyte-rich infiltrate with no signs of malignancy. A positron emission tomography (PET) scan was done to look for a primary lesion elsewhere that could have caused intraocular metastasis. The PET scan showed marginal uptake in cervical and supraclavicular lymph nodes. Cervical lymph node biopsy was done and sent for histopathologic examination (HPE). HPE showed reactive hyperplasia with few pigment-laden macrophages with no evidence of malignancy. Patient was given the option of fine needle aspiration biopsy of the ocular lesion to arrive at a diagnosis. However, in view of poor visual potential in that eye, the patient opted for enucleation.

The enucleated specimen was sent for HPE. Initial HPE report was diffuse malignant melanoma of ciliary body and choroid with mixed epithelioid and spindle cells. In view of strong clinical suspicion, reassessment of the specimen and a second opinion was sought from another laboratory. The second pathologist picked up diffuse infiltration of the ciliary body with small, round, blue cells with high mitotic activity extending into choroid and iris [Figure 3]a. Immunohistochemistry panel was done. HMB45 and S100, the melanoma markers, were negative [Figure 3]b and [Figure 3]c. Positive lymphoma markers, CD20, BCL2, and PAX5, with a Ki-67 index of 70%–80% clinched the diagnosis of diffuse large B-cell lymphoma (DLBCL) [Figure 3]d, [Figure 3]e, [Figure 3]f.

Figure 3: (a) Small, round, blue cells on HPE; (b) negative for HMB45; (c) negative for S100; (d) positive for CD20; (e) positive for BC2; (f) positive for PAX5. HPE = histopathologic examination

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In view of extremely rare incidence of primary ciliary body lymphoma, the patient underwent a repeat systemic oncology evaluation, which did not show a primary focus of tumor elsewhere in the body. The patient underwent six cycles of adjuvant chemotherapy with Rituximab, Cyclophosphamide, Hydroxydaunorubicin, Vincristine (Oncovin), Prednisone (R-CHOP) regimen and is being closely followed up under the care of the oncologist.

Discussion

Intraocular lymphomas can arise in various parts of the eye manifesting in numerous ways, but they are very rare and together form less than 1% of all intraocular tumors.^[6] Primary vitreoretinal lymphomas (PVRL) account for most intraocular lymphomas. Uveal lymphomas, on the other hand, are less common and could be either primarily arising from the uvea or secondary from a systemic lymphoma. The origin of primary uveal lymphomas is commonly the choroid, followed by the iris, making ciliary body lymphomas the least common variety.^[7] Choroidal lymphomas are usually of low grade and are morphologically similar to extranodal marginal zone lymphoma (EMZL), while PVRL are commonly of DLBCL type.^[7]

Our patient had a primary DLBCL of the ciliary body, which is very rare and has a worse prognosis compared to the EMZL type. The average 5-year disease-specific survival rate of DLBCL is 55% in contrast to 97% in EMZL.^[8] Also, high Ki-67 expression in DLBCL, which signifies the high proliferative activity of lymphoma, is associated with poor outcomes.^[9] Hence, the treating oncologist decided to administer six cycles of adjuvant chemotherapy.

The patient did not want further evaluation and treatment when there was diagnostic dilemma and opted for enucleation in view of old age and guarded visual prognosis. We definitely wanted to salvage the eye and possibly vision. Our plan was to do a fine needle aspiration biopsy of the lesion and HPE. HPE with the aid of immunohistochemistry would have given us the diagnosis of lymphoma, which could have been treated with systemic chemotherapy and then local treatment if necessary. This could have been followed by cataract surgery. So, eye salvage was definitely possible and vision salvage could have been attempted; however, visual prognosis would be guarded.

Conclusion

Our report shows that primary lymphoma of ciliary body may be considered as a differential in cases with atypical features. Histopathology and immunohistochemistry are valuable tools in diagnosing challenging cases.

Acknowledgement

Dr. S. Krishnakumar, MD Pathology, Sri Nathella Sampathu Chetty Clinical Laboratory, Chennai, is acknowledged for his technical assistance.

Confidentiality statement

Informed consent has been obtained from the patient to publish the case report and the images. All measures to protect patient's confidentiality have been taken. It has been ensured that none of the images – clinical or radiological – reveal patient's identity in any manner. Institutional review board has approved this case report vide approval number SEH/BLR/EC/2022/74.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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