Conjunctival Lymphoma

Evidence-based clinical reference for conjunctival lymphoma — the most common primary ocular adnexal malignancy. Covers MALT lymphoma biology, the salmon-pink sign, systemic staging workup, radiotherapy, and the optometrist's role in early detection and urgent referral.

Last updated: March 2026

Bulbar conjunctival MALT lymphoma. Painless salmon-pink subconjunctival mass with smooth lobulated surface and mild overlying vascular injection.

Diffuse forniceal infiltration. More advanced MALT lymphoma showing superior forniceal and nasal bulbar spread; the characteristic "fish-flesh" colouration remains visible.

Conjunctival lymphoma is the most common primary ocular adnexal malignancy, accounting for approximately 25–35% of all ocular adnexal lymphomas. It arises from lymphoid tissue within the conjunctival stroma and is almost exclusively of B-cell lineage. The vast majority (approximately 75%) represent extranodal marginal zone B-cell lymphoma (EMZBL), also termed MALT (mucosa-associated lymphoid tissue) lymphoma, which behaves in an indolent fashion.

The hallmark presentation is a painless, salmon-pink or "fish-flesh" coloured subconjunctival mass, most commonly arising from the inferior fornix or bulbar conjunctiva. Bilateral involvement occurs in approximately 20–30% of cases. Despite its benign appearance, systemic lymphoma is identified on staging workup in 20–30% of patients at presentation, emphasising that biopsy and systemic staging are mandatory for all confirmed cases.

The median age at presentation is the sixth to seventh decade; conjunctival lymphoma is rare in children outside an immunocompromised context. Management of localised disease is principally with external beam radiotherapy, achieving high local control rates. Systemic or recurrent disease requires haematological oncology involvement with rituximab-based chemotherapy regimens.

Chlamydia psittaci

A landmark 2004 Italian study (Ferreri et al.) demonstrated Chlamydia psittaci DNA in a majority of ocular adnexal MALT lymphoma specimens by PCR, implicating chronic bacterial antigen stimulation as a driver of malignant B-cell clonal expansion. Subsequent studies have shown significant geographic variation in C. psittaci prevalence (high in Italy and Austria, lower in the United States and Asia), suggesting regional cofactors. Antibiotic eradication of C. psittaci with doxycycline has produced objective tumour regressions in several series, supporting a causal role in at least a subset of cases.

Other Infectious Agents

Helicobacter pylori — the prototypic driver of gastric MALT lymphoma — has been identified in some ocular adnexal cases. Hepatitis C virus (HCV) infection has been associated with B-cell lymphoproliferative disorders at multiple sites including the conjunctiva, particularly in HCV-endemic regions. Less commonly, Borrelia burgdorferi has been implicated in European case series.

Immune Dysregulation

Chronic autoimmune conditions — particularly Sjögren syndrome, rheumatoid arthritis, and systemic lupus erythematosus — carry an elevated risk of MALT lymphoma at multiple mucosal sites due to sustained lymphoid hyperplasia and chronic antigen stimulation. Immunosuppression from solid organ transplantation, HIV/AIDS, or prolonged corticosteroid use creates a permissive environment for lymphoid neoplasia.

Idiopathic

In a substantial proportion of cases, no identifiable infectious or autoimmune trigger is found. Accumulated somatic mutations in B-cell regulatory genes (e.g., TNFAIP3, CARD11) and chromosomal translocations — particularly t(11;18)(q21;q21) involving API2-MALT1 — have been identified in MALT lymphoma and may arise spontaneously in the absence of chronic antigen drive.

The conjunctiva contains organised mucosa-associated lymphoid tissue (MALT), which normally serves as a local immune surveillance system. Under conditions of persistent antigenic stimulation — whether from chronic infection, autoimmune activity, or aberrant immune regulation — this tissue undergoes reactive lymphoid hyperplasia that can progress through successive steps to overt malignancy.

Clonal B-cell Expansion

Persistent antigen stimulation drives repeated activation of marginal zone B cells. Over time, genomic instability leads to acquisition of somatic mutations and chromosomal abnormalities that confer a survival and proliferative advantage to a single B-cell clone. The clone escapes normal apoptotic checkpoints and accumulates as a monoclonal B-cell population within the conjunctival stroma.

Key Molecular Events in MALT Lymphoma

t(11;18)(q21;q21) API2-MALT1: the most common translocation, producing a fusion oncoprotein that constitutively activates NF-κB signalling; associated with antibiotic-refractory disease
t(14;18)(q32;q21) IGH-MALT1: overexpresses MALT1 via immunoglobulin heavy chain promoter; less common
TNFAIP3 (A20) deletion: loss of this NF-κB negative regulator is common in ocular adnexal MALT lymphoma
NF-κB pathway: constitutive activation promotes lymphocyte survival, proliferation, and resistance to apoptosis across multiple MALT lymphoma subtypes

Progression to Systemic Disease

Marginal zone lymphoma may disseminate haematogenously or via lymphatics to regional lymph nodes and distant MALT sites (stomach, lung, salivary gland). Histological transformation to diffuse large B-cell lymphoma (DLBCL) represents a more aggressive evolutionary step, occurring in approximately 5–10% of MALT lymphomas, with acquisition of additional genetic hits including MYC rearrangement and TP53 mutation.

WHO Histological Subtypes

Subtype	Frequency	Behaviour	Key Feature
Extranodal Marginal Zone B-cell (MALT)	~75%	Indolent	CD20+, CD5−, CD10−; t(11;18) common
Follicular Lymphoma	~10%	Indolent	CD10+, BCL-2+; t(14;18) IGH-BCL2
Diffuse Large B-cell Lymphoma (DLBCL)	~8%	Aggressive	CD20+; rapid growth, systemic spread common
Mantle Cell Lymphoma	~4%	Aggressive	CD5+, cyclin D1+; t(11;14); systemic disease typical
Other (NK/T-cell, Burkitt, lymphoplasmacytic)	<5%	Variable	Rare; often secondary to systemic disease

Ann Arbor Staging (Modified for Ocular Adnexal Lymphoma)

Stage IE: disease confined to one extranodal site (conjunctiva ± other ocular adnexa unilaterally)
Stage IIE: extranodal site plus regional lymph node involvement (e.g. preauricular, cervical nodes)
Stage IIIE: involvement of lymph nodes on both sides of the diaphragm
Stage IV: disseminated extranodal involvement (bone marrow, liver, contralateral ocular adnexa)

The majority of conjunctival lymphomas present at Stage IE or IIE. A modified TNM classification (T1–T4a/b) has been proposed by the AJCC/UICC specifically for ocular adnexal lymphoma to capture anatomical extent more precisely.

Infectious / Inflammatory

Chlamydia psittaci infection (geographic variation)
Hepatitis C virus (HCV) infection
Helicobacter pylori infection
Borrelia burgdorferi (European series)
Chronic conjunctival inflammatory disease
Sjögren syndrome

Immune and Systemic

HIV/AIDS (particularly aggressive subtypes)
Solid organ transplantation (immunosuppression)
Prior systemic lymphoma or haematological malignancy
Rheumatoid arthritis / SLE
Prolonged systemic corticosteroid or immunosuppressant therapy

Demographic

Age over 50 years (peak: sixth to seventh decade)
Female predominance in some series (MALT subtype)
Asian ethnicity (higher incidence of ocular adnexal lymphoma)

Genetic

t(11;18)(q21;q21) API2-MALT1 translocation
TNFAIP3 (A20) biallelic inactivation
Family history of lymphoma

Pathognomonic Sign

The characteristic salmon-pink or "fish-flesh" coloured subconjunctival mass is the hallmark of conjunctival MALT lymphoma. This distinctive colouration reflects the dense, vascularised lymphoid infiltrate beneath the conjunctival epithelium and is virtually diagnostic in the appropriate clinical context.

Biomicroscopic Features

Location: inferior or superior fornix most common; bulbar conjunctiva (nasal > temporal); less commonly palpebral
Surface: smooth, lobulated, non-keratinised epithelium overlying the mass; no surface ulceration (differentiates from OSSN)
Mobility: mass moves freely over the globe, consistent with subconjunctival rather than episcleral location
Feeder vessels: fine overlying or surrounding vascularity; does not display sentinel vessels typical of OSSN or pterygium
Bilateral involvement: present in 20–30%; both eyes should be examined at every visit
Conjunctival folding: larger lesions may cause conjunctival redundancy or chemosis
Ptosis or proptosis: if orbital extension occurs
Palpable preauricular lymphadenopathy: indicates locoregional nodal involvement (Stage IIE)

Conjunctival lymphoma is characteristically asymptomatic or minimally symptomatic, which contributes to delayed presentation. The most common complaint is a visible swelling or redness noticed incidentally by the patient or during routine eye examination.

Painless visible mass: the predominant presenting complaint; often described as a "pink lump" under the eyelid
Mild foreign body sensation: particularly with larger forniceal lesions mechanically irritating the corneal surface
Epiphora (watering): if the mass obstructs the lacrimal punctum or alters lid position
Redness: mild conjunctival injection overlying the mass or from reactive hyperaemia
Ptosis or lid heaviness: with superior tarsal or forniceal involvement
Diplopia or proptosis: late symptom indicating orbital invasion; warrants urgent assessment
Constitutional symptoms (B symptoms): fever, drenching night sweats, unexplained weight loss >10% — indicate systemic lymphoma; prompt urgent haematological referral

Clinical note: Absence of pain and inflammation is a key differentiating feature from infective or allergic conjunctivitis. Any painless salmon-pink mass, particularly in a patient over 50, should be treated as lymphoma until proven otherwise.

Orbital Invasion

Extension posteriorly through Tenon's capsule into the orbital fat represents the most significant local complication, producing proptosis, restricted motility, and diplopia. Orbital invasion upgrades disease stage and necessitates more aggressive treatment with expanded radiation fields or systemic chemotherapy.

Systemic Dissemination

Approximately 20–30% of patients have concurrent systemic lymphoma at presentation, and an additional 15–20% develop systemic disease over the follow-up period, particularly those with bilateral conjunctival involvement. The most common sites of spread are regional lymph nodes, bone marrow, lung, and other MALT sites (stomach, salivary gland, thyroid).

Histological Transformation

Transformation from indolent MALT lymphoma to aggressive DLBCL occurs in 5–10% of cases and is associated with rapid lesion growth, constitutional symptoms, and markedly worsened prognosis. Early transformation may be suggested by a change in growth rate or the appearance of systemic B symptoms.

Treatment-Related Complications

Radiotherapy: dry eye syndrome (lacrimal gland irradiation), cataract, radiation retinopathy/optic neuropathy at higher doses, keratoconjunctivitis sicca
Rituximab (subconjunctival): local injection site reactions, chemosis; systemic administration carries infusion reaction risk
Recurrence: local recurrence after radiotherapy occurs in approximately 5–15%; systemic recurrence warrants restaging and chemotherapy review

Conjunctival lymphoma must always be considered a potential manifestation of systemic lymphoproliferative disease. Systemic evaluation is mandatory regardless of the apparent localisation of the ocular lesion.

Systemic Lymphoma

The conjunctiva may be the initial presenting site of systemic non-Hodgkin lymphoma. Conversely, patients with known systemic lymphoma may develop secondary conjunctival involvement. Subtypes with high rates of concurrent systemic disease at presentation include mantle cell lymphoma (nearly always systemic) and DLBCL (~50% systemic at presentation). Even MALT lymphoma — though typically localised — is associated with systemic disease in 20–30% of cases.

Sjögren Syndrome

Sjögren syndrome carries a 6- to 44-fold increased risk of developing non-Hodgkin lymphoma, predominantly MALT lymphoma. The chronically activated salivary and lacrimal gland lymphocytes serve as a fertile substrate for lymphomagenesis. Patients with Sjögren syndrome presenting with new conjunctival or periocular masses require biopsy and lymphoma workup.

HIV/AIDS

Immunodeficiency from HIV facilitates Epstein–Barr virus (EBV)-driven lymphoproliferative disease. In this population, conjunctival lymphoma tends to be more aggressive (DLBCL or EBV-positive large cell lymphoma), bilateral, and associated with systemic disease. Highly active antiretroviral therapy (HAART) has substantially reduced the incidence but not eliminated risk.

Rheumatoid Arthritis and Other Autoimmune Conditions

Chronic immune activation in rheumatoid arthritis, SLE, and vasculitis sustains B-cell hyperplasia that may undergo malignant transformation. Immunosuppressant therapies (methotrexate, azathioprine, biologics) used in these conditions may additionally impair immune surveillance of lymphoma clones.

Hepatitis C Virus

HCV drives B-cell proliferation via interaction with CD81 and the B-cell receptor, predisposing to marginal zone and lymphoplasmacytic lymphoma. Antiviral therapy with direct-acting antivirals (DAAs) has produced lymphoma regressions in HCV-associated cases analogous to the antibiotic effect in C. psittaci-driven disease.

Important: Tissue biopsy with histopathological and immunohistochemical analysis is the only definitive method of diagnosis. Clinical appearance alone, however characteristic, cannot distinguish lymphoma from reactive lymphoid hyperplasia or other masqueraders.

Clinical Assessment

Slit-lamp examination documenting lesion location, size, colour, surface characteristics, and vascularity
Fundus examination using approved non-dilating diagnostic equipment to assess posterior segment
Palpation for preauricular and cervical lymphadenopathy
Contralateral eye examination for bilateral involvement
Baseline best-corrected visual acuity and intraocular pressure

Incisional or Excisional Biopsy

Fresh tissue (not formalin-fixed) is required for flow cytometry. The biopsy specimen is processed for:

Haematoxylin and eosin (H&E): architecture, cell morphology, mitotic rate
Immunohistochemistry (IHC): CD20, CD3, CD5, CD10, CD23, BCL-2, BCL-6, cyclin D1, Ki-67 proliferation index
Flow cytometry: B-cell clonality (kappa/lambda light chain restriction), surface immunophenotype
FISH / cytogenetics: detection of t(11;18), t(14;18), t(11;14) and other translocations
PCR for clonality: IGH gene rearrangement analysis; PCR for C. psittaci DNA in relevant populations

Systemic Staging Workup

CT chest/abdomen/pelvis (with contrast) or PET-CT: gold standard for nodal and extranodal staging; PET-CT preferred for aggressive subtypes
Bone marrow biopsy: recommended for DLBCL, mantle cell, and follicular lymphoma; selectively for MALT depending on clinical stage
MRI orbits: delineates orbital extension and involvement of lacrimal gland, extraocular muscles, and optic nerve
Full blood count and blood film: leukaemic phase or cytopenias
LDH, beta-2 microglobulin, uric acid: tumour burden markers and risk stratification
Hepatitis B, C, HIV serology: mandatory before rituximab therapy (HBV reactivation risk)
Serum immunoelectrophoresis: M-protein in lymphoplasmacytic variants

Imaging of the Ocular Adnexa

Anterior segment OCT (AS-OCT): reveals subepithelial hyporeflective infiltrate with intact overlying epithelium, helping distinguish lymphoma from OSSN (which shows hyperreflective thickened epithelium)
Ultrasound biomicroscopy (UBM): characterises anterior extension and depth
B-scan ultrasonography: assesses orbital component if orbital invasion is suspected

Singapore Optometry Scope Note: Optometrists in Singapore play a critical role in early detection of suspicious conjunctival masses. Upon identifying a salmon-pink or atypical subconjunctival lesion, optometrists should document findings with anterior segment photography and refer urgently to ophthalmology. Optometrists cannot prescribe topical corticosteroids, NSAIDs, or chemotherapeutic agents. Posterior segment assessment should be performed using approved non-dilating diagnostic equipment; dilated fundus examination is not within the Singapore optometry scope of practice. All management beyond observation and urgent referral — including biopsy, staging, radiotherapy, and systemic treatment — is provided by ophthalmology and haematological oncology.

1. Urgent Referral and Biopsy

Any suspected conjunctival lymphoma must be referred promptly to an ophthalmologist for incisional or excisional biopsy. Delay in biopsy risks disease progression and delayed initiation of staging and treatment. Fresh tissue handling is essential to preserve flow cytometry and FISH analysis capability.

2. Localised Disease — External Beam Radiotherapy (EBRT)

EBRT is the gold standard for localised (Stage IE) conjunctival lymphoma, achieving local control rates of 90–98%. Indolent subtypes (MALT, follicular) require lower doses than aggressive subtypes.

Subtype	Dose	Fractions	Field
MALT / Follicular (indolent)	24–30 Gy	12–15	Involved field; lens shield used
DLBCL (aggressive)	36–40 Gy	18–20	Extended field; +/− systemic R-CHOP

Modern lens-sparing techniques (e.g. intensity-modulated radiotherapy, proton therapy) reduce the risk of radiation-induced cataract and lacrimal gland damage.

3. Antibiotic Therapy (Chlamydia psittaci–Associated Cases)

Antibiotic treatment for C. psittaci eradication is prescribed by an ophthalmologist or infectious disease physician. Optometrists in Singapore cannot prescribe systemic antibiotics for this indication.

Doxycycline 100 mg twice daily for 3 weeks — first-line for C. psittaci-positive MALT lymphoma
Objective response rates of 20–65% depending on geographic population and C. psittaci prevalence
May be offered as initial treatment before radiotherapy in confirmed C. psittaci-positive, Stage IE cases
HCV-associated cases: direct-acting antiviral therapy may produce lymphoma regression (manage with hepatology)

4. Systemic Immunochemotherapy (Managed by Haematological Oncology)

All systemic treatment is initiated and monitored by haematological oncology. Optometrists should not independently prescribe or manage any of the following agents.

Rituximab (anti-CD20): systemic IV infusion for disseminated MALT, follicular, or DLBCL; subconjunctival injection has been reported in small series for localised disease
R-CHOP (rituximab + cyclophosphamide, doxorubicin, vincristine, prednisolone): standard of care for DLBCL and aggressive lymphoma
R-CVP or chlorambucil: for disseminated indolent lymphoma with systemic involvement
Watch-and-wait: selected asymptomatic, very low-burden Stage IV indolent lymphoma under haematology supervision

5. Surveillance After Treatment

Slit-lamp examination every 3–6 months for 2 years, then annually
Anterior segment photography for comparison at each visit
CT or PET-CT restaging at 3 months post-treatment and if recurrence is suspected
Annual full blood count, LDH, and clinical lymphadenopathy assessment
Monitor for radiotherapy sequelae: dry eye, cataract (refer to ophthalmologist)

MALT Lymphoma (Indolent)

Localised conjunctival MALT lymphoma carries an excellent prognosis with appropriate treatment. External beam radiotherapy achieves local control in over 90% of Stage IE disease, with 5-year overall survival rates of approximately 85–95%. Disease-specific survival approaches 100% for truly localised MALT lymphoma. However, systemic recurrence can occur years to decades after initial treatment, necessitating lifelong surveillance.

Follicular Lymphoma

Localised follicular lymphoma treated with radiotherapy has 5-year progression-free survival of approximately 65–75%. Systemic recurrence is more common than in MALT lymphoma; long-term outcomes depend heavily on systemic disease burden.

DLBCL (Aggressive)

DLBCL carries a substantially worse prognosis. With combined chemo-immunotherapy (R-CHOP) and consolidation radiotherapy, approximately 50–60% of patients achieve long-term remission. International Prognostic Index (IPI) score is the primary risk stratification tool; high-IPI DLBCL has a 5-year survival of approximately 25–35% despite aggressive therapy.

Prognostic Factors

Favourable

Stage IE at diagnosis
MALT or follicular histology
Low Ki-67 proliferation index
t(11;18) negative (antibiotic responsive)
Unilateral disease

Unfavourable

Stage IIE–IV at diagnosis
DLBCL or mantle cell histology
Histological transformation
Bilateral involvement
Constitutional B symptoms
Elevated LDH or beta-2 microglobulin

Condition	Key Distinguishing Features
Reactive Lymphoid Hyperplasia	Polyclonal B-cell proliferation; identical clinical appearance to MALT lymphoma; only biopsy with clonality testing distinguishes the two
OSSN (Ocular Surface Squamous Neoplasia)	Gelatinous/leukoplakic limbal mass with sentinel vessels; involves epithelium (AS-OCT shows hyperreflective thickened epithelium); no salmon-pink hue
Conjunctival Amyloidosis	Waxy, yellowish deposits in fornix or caruncle; associated with local or systemic amyloid; Congo red stain positive on biopsy
Conjunctival Melanoma / PAM	Pigmented lesion; variable colouration; more nodular; may arise from PAM with atypia; biopsy essential
Episcleritis / Scleritis	Redness and dilated episcleral vessels; scleritis causes severe deep pain; no mass; blanches with phenylephrine (episcleritis)
Orbital Cellulitis	Acute onset, fever, lid oedema, painful proptosis; no discrete subconjunctival mass; systemic signs of infection
Idiopathic Orbital Inflammation (Pseudotumour)	Painful proptosis, diplopia; CT shows diffuse orbital infiltration; responds to systemic corticosteroids; biopsy distinguishes from lymphoma
Dermoid / Lipodermoid Cyst	Typically presents in childhood; yellowish, smooth, mobile; located at superotemporal limbus; no salmon-pink colour
Carcinoid Metastasis	Rare; pink subconjunctival deposits from haematogenous spread; history of systemic carcinoid; serotonin markers positive

The salmon-pink sign: a painless, salmon-pink or fish-flesh subconjunctival mass in a patient over 50 is lymphoma until proven otherwise — biopsy is mandatory even if the lesion has been present for years.
Biopsy handling matters: fresh tissue (not formalin-fixed) must be sent for flow cytometry alongside formalin-fixed tissue for immunohistochemistry. Incorrect tissue handling can render the specimen non-diagnostic.
Clinical appearance cannot exclude reactive hyperplasia: reactive lymphoid hyperplasia is histologically benign but indistinguishable from MALT lymphoma by slit-lamp appearance alone; clonality testing by flow cytometry or PCR is the deciding investigation.
Systemic staging is always mandatory: even an apparently isolated conjunctival lesion requires CT chest/abdomen/pelvis or PET-CT — 20–30% of patients have concurrent systemic lymphoma at presentation.
AS-OCT is a useful non-invasive adjunct: MALT lymphoma shows intact overlying epithelium with a hyporeflective subepithelial infiltrate — in contrast to OSSN, which shows hyperreflective epithelial thickening with an abrupt border at the normal epithelium transition.
Bilateral examination is essential: up to 30% of conjunctival lymphoma is bilateral at diagnosis; the second eye may be missed if not systematically examined, leading to understaging.
Geographic variation in C. psittaci: doxycycline eradication therapy is most likely to produce a response in European patients where C. psittaci prevalence is high; in Singapore and Southeast Asia, the association is weaker and radiotherapy remains first-line for confirmed lymphoma.
Lifelong surveillance: indolent lymphomas (MALT, follicular) can recur years or decades after successful local treatment; annual review with anterior segment photography is the standard of care even after apparent complete remission.
Mantle cell lymphoma is a red flag: unlike MALT lymphoma, mantle cell lymphoma presenting at the conjunctiva is nearly always a manifestation of widespread systemic disease and carries a substantially worse prognosis; urgent haematology referral is critical.

Shields CL, Shields JA, Carvalho C, Rundle P, Smith AF. Conjunctival lymphoid tumors: clinical analysis of 117 cases and relationship to systemic lymphoma. Ophthalmology. 2001;108(5):979-84.
Ferreri AJ, Guidoboni M, Ponzoni M, De Conciliis C, Dell'Oro S, Fleischhauer K, et al. Evidence for an association between Chlamydia psittaci and ocular adnexal lymphomas. J Natl Cancer Inst. 2004;96(8):586-94.
Coupland SE, Damato B. Understanding intraocular lymphomas. Clin Experiment Ophthalmol. 2008;36(6):564-78.
Ferry JA, Fung CY, Zukerberg L, Lucarelli MJ, Hasserjian RP, Preffer FI, et al. Lymphoma of the ocular adnexa: a study of 353 cases. Am J Surg Pathol. 2007;31(2):170-84.
Stefanovic A, Lossos IS. Extranodal marginal zone lymphoma of the ocular adnexa. Blood. 2009;114(3):501-10.
Sjö LD. Ophthalmic lymphoma: epidemiology and pathogenesis. Acta Ophthalmol. 2009;87 Thesis 1:1-20.
Raderer M, Wohrer S, Streubel B, Troch M, Turetschek K, Jager U, et al. Assessment of disease dissemination in gastric compared with extragastric mucosa-associated lymphoid tissue lymphoma using extensive staging: a single-center experience. J Clin Oncol. 2006;24(19):3136-41.
Meunier J, Lumbroso-Le Rouic L, Vincent-Salomon A, Dendale R, Asselain B, Arnaud P, et al. Ophthalmologic and intraocular non-Hodgkin's lymphoma: a large single centre study of initial characteristics, natural history, and prognostic factors. Hematol Oncol. 2004;22(4):143-58.
Decaudin D, de Cremoux P, Vincent-Salomon A, Dendale R, Rouic LL. Ocular adnexal lymphoma: a review of clinicopathologic features and treatment options. Blood. 2006;108(5):1451-60.
Rasmussen PK, Ralfkiaer E, Prause JU, Sjo LD, Kamper P, d'Amore F, et al. Diffuse large B-cell lymphoma of the ocular adnexa: a nation-based study. Acta Ophthalmol. 2013;91(2):163-9.
Bolek TW, McLaughlin MP, Moyses J, Copeland EM 3rd, Glatfelter AA, Marcus RB Jr. Radiotherapy in the management of orbital lymphoma. Int J Radiat Oncol Biol Phys. 1999;44(1):31-6.
Uno T, Isobe K, Shikama N, Nishikawa A, Oguchi M, Ueno N, et al. Radiotherapy for extranodal, marginal zone, B-cell lymphoma of mucosa-associated lymphoid tissue originating in the ocular adnexa: a multiinstitutional, retrospective review of 50 patients. Cancer. 2003;98(4):865-71.
Eich HT, Sommer K, Eich TS, Staar S, Zander T, Engert A, et al. Low-dose orbital and periorbital irradiation for indolent orbital and conjunctival lymphomas: long-term results, visual outcome, and toxicity. Int J Radiat Oncol Biol Phys. 2009;75(4):1083-9.
Govi S, Dognini GP, Licata G, Messina C, Vitolo U, Martelli M, et al. Six-month oral clarithromycin regimen is safe and active in extranodal marginal zone lymphomas: final results of a single-centre phase II trial. Br J Haematol. 2010;150(2):226-9.
Ponzoni M, Ferreri AJ, Guidoboni M, Lettini AA, Seregard S, Freschi M, et al. Chlamydia psittaci infection and ocular adnexal lymphomas: a putative etiological correlation. Adv Anat Pathol. 2012;19(4):261-5.
Ben Simon GJ, Cheung N, McKelvie P, Fox R, McNab AA. Oral chlorambucil for extranodal, marginal zone, B-cell lymphoma of mucosa-associated lymphoid tissue of the orbit. Ophthalmology. 2006;113(7):1209-13.
Coupland SE, White VA, Rootman J, Damato B, Finger PT. A TNM-based clinical staging system of ocular adnexal lymphomas. Arch Pathol Lab Med. 2009;133(8):1262-7.
McKelvie PA. Ocular adnexal lymphomas: a review. Adv Anat Pathol. 2010;17(4):251-61.
Graue GF, Finger PT, Maher EA, Della Rocca DA, Della Rocca RC, Jhanwar S. Ocular adnexal lymphoma at the ocular surface. Ocul Oncol Pathol. 2017;3(1):1-14.
Hong JW, Kim HJ, Roh JH, Khwarg SI, Woo KI, Kim YD. Conjunctival lymphoma in Korea: a review of 30 cases. Korean J Ophthalmol. 2019;33(6):503-11.

Disclaimer: This guide is for educational purposes and clinical reference. Always exercise professional judgment and follow local regulations and scope of practice guidelines. Refer to ophthalmology when appropriate for surgical management or complex cases.

Illustration

Overview

Etiology