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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 2  |  Issue : 3  |  Page : 767-769

An orbital mass presenting as an eyelid lesion


Department of Orbit and Oculoplasty, Aswini Eye Clinic, Amrit Hospital, Chennai, Tamil Nadu, India

Date of Submission27-Dec-2021
Date of Acceptance12-Apr-2022
Date of Web Publication16-Jul-2022

Correspondence Address:
Dr. Jeevitha Jagannathan
8, Gangai Amman Kovil Street, Sri Devi Kuppam, Valasaravakkam, Chennai - 600 087, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_3188_21

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  Abstract 


A 45-year-old man presented with history of painless, slow-growing mass in the medial aspect of right lower eyelid over 2 years. On examination, the mass was noted to be firm, mobile, and nontender. On excision biopsy, histopathologic and immunohistochemistry report was consistent with low-grade non-Hodgkin's B-cell lymphoma. He was then treated with local radiotherapy. Using 6 MeV electrons, a dose of 24 Gy was delivered in 12 cycles over a period of 2 weeks to the right lower lid, with a centimeter bolus. Though non-Hodgkin's lymphoma of the ocular adnexa is rare, it should be considered in differential diagnosis, as they have the potential for rapid systemic involvement and may need treatment that is different from that of other common diseases at the same site.

Keywords: Eyelid Lesion, extranodal marginal zone lymphoma, non-Hodgkin's lymphoma, orbital mass


How to cite this article:
Jagannathan J, Murugesan AP, Kumar S L, Kumar S R. An orbital mass presenting as an eyelid lesion. Indian J Ophthalmol Case Rep 2022;2:767-9

How to cite this URL:
Jagannathan J, Murugesan AP, Kumar S L, Kumar S R. An orbital mass presenting as an eyelid lesion. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Aug 11];2:767-9. Available from: https://www.ijoreports.in/text.asp?2022/2/3/767/351178



A painless, slow-growing mass under the eyelid is a diagnostic dilemma. Lymphoma of the ocular adnexa (OAL) is a heterogeneous group of malignancies, accounting for approximately 1%–2% of non-Hodgkin's lymphomas, 8% of extranodal lymphomas,[1] and 2%–11% of orbital tumors.[2],[3],[4] Ocular adnexal lymphomas are mostly seen in the fifth to seventh decade of life with female predominance.[5] The most frequently involved site is the orbit (40%), followed by conjunctiva (35%–40%), lacrimal gland (10%–15%), and eyelid (10%).[4] Although a variety of lymphoma types constitutes OAL, most of them are non-Hodgkin's B-cell type, with less than 1%–3% being T-cell types.


  Case Report Top


A 45-year-old, otherwise healthy man presented with history of slowly progressive swelling in the medial aspect of the right lower lid [Figure 1] over 2 years, which was static in size for the last 6 months. He did not have any symptoms, except for slight discomfort and cosmetic concern. No proptosis was noted. On palpation, the mass was firm, mobile, and nontender. On ocular examination, the best corrected visual acuity was 6/6 in both eyes with a normal anterior and posterior segment. Magnetic resonance imaging (MRI) T2-weighted images showed a well-defined hyperintense lesion in the inferonasal quadrant of the right orbit, abutting the inferior rectus muscle and the globe without deforming it [Figure 2]. Clinically, it was suspected to be a variant of lipoma, and excision biopsy of the mass was planned.
Figure 1: External photograph revealing right lower eyelid swelling

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Figure 2: Magnetic resonance T2 image showing the lesion in the inferonasal quadrant of the right orbit

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Through a lower lid crease incision, after separating the orbicularis fibers, the orbital septum was opened. The medial fat pad was identified and retracted to expose the mass. The mass was well defined and firm to rubbery in consistency [Figure 3]. With meticulous soft tissue dissection, the mass was isolated from the surrounding structures. The posterior extent was found to go deeper up to the orbital rim. The entire mass was tracked back and excised completely. The mass was sent for histopathologic examination.
Figure 3: Intraoperative picture of a well-defined, firm mass

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On gross examination, 1 × 1 cm, grayish-brown soft tissue mass with gray tan cut surface was noted. Microscopic examination revealed fibroadipose tissue with nodular infiltrate of atypical small lymphoid cells with scant cytoplasm arranged in sheets admixed with areas of hyalinization. Increased mitosis and intervening vascular proliferation were noted [Figure 4]a. Immunohistochemistry was suggestive of low-grade non-Hodgkin's B-cell lymphoma, marginal zone lymphoma with CD20 diffuse strong cytoplasmic positivity, BCL-2 strong positivity, and CD45 strong cytoplasmic positivity [Figure 4]b, [Figure 4]c, [Figure 4]d.
Figure 4: (a) Histopathologic examination revealing fibroadipose tissue with nodular infiltrate of atypical small lymphoid cells (hematoxylin and eosin staining, 10×). (b) IHC at 10 × showing diffuse strong cytoplasmic positivity for CD20. (c) IHC showing BCL-2 positivity. (d) IHC showing strong cytoplasmic positivity for CD45. IHC = immunohistochemistry

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Patient was referred to an oncologist for further assessment. Laboratory investigations and imaging study including positron emission tomography (PET) revealed no other areas of involvement. He was diagnosed with primary ocular adnexal extranodal marginal zone lymphoma and treated with local radiation therapy. Using 6 MeV electrons, a dose of 24 Gy was delivered in 12 cycles over a period of 2 weeks to the right lower lid, with a centimeter bolus. Then, he was advised to be on regular follow-up. Patient was reviewed at 6 months and 1 year after completion of treatment. Now, he is on periodic follow up to observe for recurrences if any.


  Discussion Top


Primary ocular adnexal lymphoma is defined as a OAL without evidence of concurrent systemic lymphoma and with no prior history of lymphoma. The most common subtype, accounting for up to 80% of cases of primary ocular adnexal lymphoma, is marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) type. MALT lymphomas typically arise in tissues or organs that are normally devoid of any organized lymphoid tissue, such as the orbital region, but acquire reactive lymphoid tissue in response to persistent antigenic stimulation, as a result of chronic inflammatory or autoimmune disorders. The majority (85%–90%) of patients with MALT lymphoma present with localized disease (stage I).[5]

The approach to staging ocular adnexal lymphoma is similar to that for lymphoma in general and typically includes thorough clinical and laboratory examinations. Although computed tomography (CT) or magnetic resonance imaging (MRI) with contrast is valuable in determining the extent of local disease of the orbit, eyelid, and paranasal sinuses, PET may be superior for the initial staging of ocular adnexal lymphoma. When compared with CT, use of PET has upstaged a majority of patients with ocular adnexal lymphoma.[6],[7],[8]

The final treatment decision requires a multidisciplinary approach, taking into account the histopathologic type, the extent of the disease, the impact on the eye or visual function, and disease-related prognostic factors. The therapy of choice in stage I tumors is radiotherapy,[9] while disseminated disease is treated with chemotherapy. Despite the indolent course of extranodal marginal zone B-cell lymphoma, they are renowned for recurrence in extranodal sites, including the lung, salivary glands, as well as other ocular adnexal sites. Therefore, long-term follow-up with 6-month examinations is recommended. Major prognostic criteria for the ocular adnexal lymphomas include anatomic location of the tumor, stage of disease at the first presentation, lymphoma subtype as determined using the revised european american lymphoma classification (REAL) classification, immunohistochemical markers, and determining factors such as the tumor growth rate and serum lactate dehydrogenase level.[10]


  Conclusion Top


This case was reported for its clinical presentation as an eyelid mass at an early age. In conclusion, when confronted with an unusual ocular adnexal mass, a diagnostic biopsy should be performed and non-Hodgkin's lymphoma should be considered. This is essential because the diagnosis changes the treatment modality. Surgical excision, usually performed for more common ocular adnexal tumors, is not successful in such patients. If not diagnosed correctly, orbital lymphoma may lead to dissemination of the disease. Patients who present with isolated orbital disease should be re-examined periodically for systemic disease.

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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Isaacson PG, Wright D. Malignant lymphoma of mucosa-associated lymphoid tissue: A distinctive type of B-cell lymphoma. Cancer 1983;52:1410-6.  Back to cited text no. 1
    
2.
Kim UR, Khazaei H, Stewart WB, Shah AD. Spectrum of orbital disease in South India: An aravind study of 6328 consecutive patients. Ophthal Plast Reconstr Surg 2010;26:315-22.  Back to cited text no. 2
    
3.
Shields JA, Bakewell B, Augsburger JJ, Flanagan JC. Classification and incidence of space-occupying lesions of the orbit: A survey of 645 biopsies. Arch Ophthalmol 1984;102:1606-11.  Back to cited text no. 3
    
4.
Moslehi R, Devesa SS, Schairer C, Fraumeni JF Jr. Rapidly increasing incidence of ocular non-Hodgkin lymphoma. J Natl Cancer Inst 2006;98:936-9.  Back to cited text no. 4
    
5.
Stefanovic A, Lossos IS. Extranodal marginal zone lymphoma of the ocular adnexa. Blood 2009;114:501-10.  Back to cited text no. 5
    
6.
Gayed I, Eskandari MF, McLaughlin P, Pro B, Diba R, Esmaeli B. Value of positron emission tomography in staging ocular adnexal lymphomas and evaluating their response to therapy. Ophthalmic Surg Laser Imaging 2007;38:319-25.  Back to cited text no. 6
    
7.
Sullivan TJ, Valenzuela AA. Imaging features of ocular adnexal lymphoproliferative disease. Eye (Lond) 2006;20:1189-95.  Back to cited text no. 7
    
8.
Valenzuela AA, Allen C, Grimes D, Wong D, Sullivan TJ. Positron emission tomography in the detection and staging of ocular adnexal lymphoproliferative disease. Ophthalmology. 2006;113:2331-7.  Back to cited text no. 8
    
9.
Masanari N, Satoshi I, Tatekawa K, Takama N, Miyakawa A, Kubota T, et al. Radiotherapy alone for stage IE ocular adnexal mucosa-associated lymphoid tissue lymphomas: Long-term results. Radiat Oncol 2020;15:25.  Back to cited text no. 9
    
10.
Coupland SE, Hummel M, Stein H. Ocular adnexal lymphomas: Five case presentations and a review of the literature. Surv Ophthalmol 2002;47:470-90.  Back to cited text no. 10
    


    Figures

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



 

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