Conjunctival Lymphoma Research Articles

Lymphoma under disguise of conjunctivitis (clinical observations)

We present two clinical cases of conjunctival lymphoma, the disease which is classified as a malignant tumor of the eye. Conjunctival lymphoma has systemic involvement. Patients presented in our monitoring study received a conservative treatment for chronic conjunctivitis over a long period of time. The article considers the diagnostics and treatment plan of lymphoproliferative eye disease, which allows an increased effectiveness of treating.

Refractory follicular conjunctival lesions: overlook as just inflammation or not?

PurposeTo report the clinical manifestations of 199 patients with suspected conjunctival lymphoma, the associations between these features and the pathological diagnoses, and the prognosis of conjunctival lesions during long-term follow-up.MethodsWe...

Conjunctival lymphomas in Japanese monozygotic twins: A case report.

An individual with a twin who has developed leukemia or non-Hodgkin lymphoma (NHL) has an increased risk of developing the same disease, particularly with monozygotic twins. The few reported pairs of twins who developed NHL had similar primary sites and pathological subtypes. Here, we present the first reported cases of primary conjunctival NHL in both female monozygotic twins. Twin 1 was diagnosed with an extranodal marginal zone lymphoma (EMZL; Ann Arbor stage IE) in the right conjunctiva at 25 years old and a subsequent tumor in the left conjunctiva at 39 years, and was also histopathologically diagnosed as EMZL. No infiltration of other organs was detected and both lesions were surgically excised. At the age of 40 years, Twin 2 was diagnosed with an EMZL (Ann Arbor stage IE) in the right conjunctiva without infiltration of other organs and was treated with external beam radiation therapy rather than surgery. Complete remission was achieved in both twins; neither developed conjunctival recurrences. This study highlights the importance of examining the other, apparently healthy twin when one twin develops conjunctival lymphoma.

Immunoglobulin G4 (IgG4)-Positive Ocular Adnexal Mucosa-Associated Lymphoid Tissue Lymphoma and Idiopathic Orbital Inflammation.

To report clinical and pathological characteristics of idiopathic orbital inflammation and ocular adnexal mucosa-associated lymphoid tissue (MALT) lymphoma with immunoglobulin G4 (IgG4)-positive plasma cells. A retrospective histopathological review and clinical case series. A total of 51 biopsy samples from January 2005 to December 2015 were used in this study, including 21 cases of biopsy-confirmed idiopathic orbital inflammation and 30 cases of biopsy-confirmed ocular adnexal MALT lymphoma. Most cases of ocular adnexal lymphoma were conjunctival tissue. Retrospective immunohistochemical studies were performed to estimate the IgG4 and IgG4/IgG ratios. Histopathologic features, demographic and clinical data, radiologic findings, treatment, and follow-up information for each patient were analyzed. Among idiopathic orbital inflammation, 6 (28.6%) of the 21 patients were diagnosed as "probable" ocular adnexal IgG4-related diseases and 13 (43.3%) of the 30 patients were diagnosed as MALT lymphoma with IgG4-positive plasma cells. Six cases of 13 IgG4-positive MALT lymphoma group had contralateral chronic inflammatory lesions infiltrated by IgG4-positive plasma cells, which was significantly (p = 0.007) higher than that in the IgG4-negative group. Conjunctival involvement was 69% of the IgG4-positive MALT lymphoma cases. Bilateral involvement of the ocular adnexa was significantly (p = 0.02) more frequent among IgG4-positive MALT lymphoma patients than that in IgG4-positive idiopathic orbital inflammation patients. Recurrence rate in the IgG4-positive group was higher (p = 0.05) than that in the IgG4-negative group but not significantly. This study presented an unusual framework of ocular adnexal IgG4-related inflammation, in conjunctiva. It is important to understand contralateral chronic inflammatory lesions and their relationship with IgG4-positive MALT lymphoma. Tissue biopsy and IgG4 immunostaining are required for all cases because IgG4-positive MALT lymphoma can arise from a pre-existing IgG4-positive chronic inflammatory lesions. This is the first study that performs IgG4 immunostaining for tissue from a relatively large number of conjunctival MALT lymphomas in a single center. Therefore, it will help to diagnose conjunctival lymphoproliferative disease.

Bilateral Conjunctival Mucosa-Associated Lymphoid Tissue Type Lymphoma in a Kidney Transplant Recipient

Bilateral Conjunctival Mucosa-Associated Lymphoid Tissue Type Lymphoma in a Kidney Transplant Recipient

Lymphome conjonctival révélant un lymphome systémique

Lymphome conjonctival révélant un lymphome systémique

Recurrent adnexal lymphoma in a horse

SummaryLymphoma is a relatively uncommon neoplasm in the horse; however, it is considered the most common neoplasia of the equine haemolymphatic system. Limited reports of adnexal lymphoma have been documented in the literature, with no known reports of recurrence nearly a decade after mass excision. The objective is to describe a case of recurrent adnexal lymphoma in a 20‐year‐old Quarter Horse gelding presented to the Ophthalmology service at the University of Florida Veterinary Hospital (UFVH) for evaluation of a solitary subconjunctival mass of the right eye (OD). A focal, pink, fleshy mass associated with the dorsomedial bulbar conjunctiva was noted. An excisional biopsy was performed. Histopathology revealed lymphoma with clean margins. The client declined staging and further therapy at the initial visit. Approximately 8 years later, the horse began to exhibit intermittent mild to moderate diffuse swelling of the conjunctiva OD. Six months after onset, the swelling worsened acutely and was accompanied by severe swelling of the left conjunctiva (OS). The horse then returned to UFVH and was diagnosed with diffuse, bilateral conjunctival lymphoma. Humane euthanasia was elected. T cell lymphoma was confirmed with immunohistochemistry performed post‐mortem. At necropsy, neoplastic tissue was observed throughout the adnexal and ocular tissues of both eyes and in the submandibular lymph nodes. Adnexal lymphoma is an uncommon neoplasm in the horse. To the authors’ knowledge, this is the first documentation of a nodular adnexal lymphoma that involved an 8‐year period without recurrence followed by development of diffuse, bilateral disease. Typically, surgical excision of the nodular form of lymphoma involving the extraocular tissues yields an improved prognosis compared with the diffuse form of the disease. Further investigation is necessary to determine if nodular forms of adnexal lymphoma represent early stages of diffuse extraocular or systemic disease.

Current Therapies in Superficial Malignant Tumors

This article is a review of diagnostic and therapeutic possibilities in common epibulbar malignant tumors, such as basal cell carcinoma, conjunctival lymphoma, squamous cell carcinoma and conjunctival melanoma. Most importantly, for every tumor patient there is a detailed anamnesis, split lamp examination and photo documentation. Further regular controls after therapy are required due to the risk of recurrences.Basal cell carcinoma is the most common tumor of the periocular skin, divided into three subtypes (nodular, superficial and morphea). The treatment consists of total excision with a sufficient safety margin.The most common tumor of the orbital and ocular adnexa are lymphoma. They can appear primary or secondary, as lymphatic system disease. The gold standard therapy is a percutaneous irradiation.Squamous cell carcinoma and its precancerous stages (CIN I - III) are frequently diagnosed on the conjunctiva. A complete tumor excision is required, but sometimes it is not possible due to large tumor growth. Therefore, an adjuvant therapy with mitomycin C eyedrops, brachytherapy with ruthenium plaque or proton radiotherapy is required.Conjunctival melanoma is a rare, but life-threatening disease. Melanosis with atypia has a high risk for degeneration and requires further treatment with mitomycin C eyedrops. In cases of malignant tumor growth, an excision with a non-touch technique and adjuvant therapy with brachy or proton irradiation should be applied.

Meibomian Gland Dysfunction Associated With Periocular Radiotherapy.

To investigate the influence of periocular radiotherapy on meibomian glands. We evaluated 28 patients (40 eyes) who received radiotherapy (RT group) for conjunctival or orbital lymphoma and 30 age-matched control subjects (60 eyes). Subjects underwent slit-lamp examination of the eyelids, Schirmer test, meibography, and evaluation of tear film breakup time (TBUT), Ocular Surface Disease Index (OSDI) scores, meibomian glands evaluation (meiboscore, meibum expressibility, and lid margin abnormality scores), and tear film lipid layer thickness using an ocular surface interferometer. These parameters were compared between subjects in the RT and control groups. Meiboscores as well as meibum expressibility and OSDI scores in the RT group were significantly higher compared with those in the control group (1.6 ± 0.9 vs. 0.4 ± 0.6, 1.6 ± 1.0 vs. 0.2 ± 0.4, and 48.1 ± 21.4 vs. 6.2 ± 4.4, respectively, P < 0.001, all), whereas the Schirmer value (9.2 ± 5.1 vs. 12.3 ± 5.2, P = 0.004), TBUT (4.2 ± 2.5 vs. 6.4 ± 2.6, P = 0.001), and lipid layer thickness (61.0 ± 29.3 vs. 85.2 ± 20.0, P < 0.001) in the RT group were lower compared with those in the control group. The percentage of meibomian gland dropout was significantly correlated with age (P = 0.025) and total radiation dose (P = 0.012), regardless of the target location of irradiation. Even low-dose irradiated eyes (<30 Gy) exhibited significantly higher meiboscores (P < 0.001) and shorter TBUT (P = 0.005) compared with control eyes. Eyes that received periocular radiotherapy exhibited relatively high tear film instability induced by meibomian gland dysfunction, contributing to the high severity of dry eye symptoms.

Characteristics of primary extranodal marginal zone B-cell lymphoma in Korea: conjunctiva versus other ocular adnexa

AimsTo compare the clinical characteristics and prognosis of primary conjunctival versus other ocular adnexal extranodal marginal zone B-cell lymphoma (EMZL).MethodsRetrospective review of clinical records for all consecutive patients with primary...

Conjunctival lymphoma during pregnancy: a case report

BackgroundTo present a case of conjunctival lymphoma in a young woman complicated by pregnancy.Case presentationA 38-year-old previously healthy woman presented with a 2-year history of progressive right blepharoptosis. Giant papillomatous sessile masses were identified in the upper and lower fornix bilaterally and involved the tarsus of the right upper lid. The remaining ophthalmic examination was unremarkable. Histopathology and immunohistochemistry showed mucosa-associated lymphoid tissue (MALT) lymphoma with immunoglobulin kappa monotype. Further workup showed no evidence of systemic lymphoma or orbital involvement.ConclusionsPartial regrowth of conjunctival lymphoma occurred 6 months after excision and the MALT lymphoma remained indolent during the course of her pregnancy without radiotherapy.

Low-Dose Radiation Therapy for Primary Conjunctival Marginal Zone B-Cell Lymphoma.

PurposeThe purpose of this study was to evaluate the clinical features and the long-term outcomes of primary conjunctival marginal zone B-cell lymphoma (MZBCL) patients who were treated with radiation therapy (RT).Materials and MethodsRetrospective data of 79 patients with 121 primary conjunctival MZBCL lesions were collected from January 1, 2001 till June 30, 2014. All lesions were treated by local RT (26 Gy) with patient-specific customized lens-shielding device.ResultsThe current Korean patients’ cohort showed younger median age at diagnosis (38 years), great female preponderance (78.5%) and more frequent bilateral involvement (53.2%) than the previous studies. Following 26 Gy’s RT, excellent clinical outcomes were achieved: 5-year rates of overall survival, local relapse-free survival, and contralateral relapse-free survival were 100%, 98.1%, and 91.5%, respectively. Two patients (2.5%) developed local relapse and five (6.3%) developed relapse at initially uninvolved contralateral conjunctiva with median interval of 52.9 months, and late adverse events of grade 2 and 3 occurred in seven (8.8%) and two (2.5%) patients, respectively.Conclusion26 Gy’s RT was highly effective and safe, with the use of lens-shielding device, in treating patients with primary conjunctival MZBCL.

Conjunctival lymphoma in right eye: Case report

Abstract Case report A 43-year-old woman presented with a salmon-colored patch of 0.7 mm diameter in the right eye that extended into the lower fornix in the bulbar and tarsal conjunctiva, with irregular edges, and highly vascularized. Incisional biopsy was performed, showing it to be a low-grade conjunctival non-Hodgkin B cell lymphoma (or a mucosa associated lymphoid tissue [MALT] lymphoma). Discussion The lesion remained stable for 24 months of follow-up, when a relapse of the condition occurred, producing an enlargement of the initial lesion. The definitive diagnosis is made by biopsy of the affected tissue and histopathologic study.

Updates in Ocular Surface Tumor Diagnostics.

Purpose To evaluate technologies and techniques available for the diagnosis of ocular surface tumors. Methods A review of the literature from 1947 to 2017, through the PubMed Database, was conducted in order to evaluate current diagnostic methods for ocular surface tumors. Results Ocular surface squamous neoplasia, conjunctival melanoma, and conjunctival lymphoma are the three most common ocular surface malignancies. Technologies available to assist with diagnosis of these conditions, in addition to full thickness biopsy, include vital dyes, aspiration and impression cytology, in vivo confocal microscopy, ultrasound biomicroscopy, genetic testing, and anterior segment optical coherence tomography. Conclusions Histology remains the gold standard for diagnosis for all 3 of these malignancies. However, multiple diagnostic techniques are available to assist in making preliminary and early diagnoses, in differentiating between similar-appearing lesions, and in some cases, avoiding biopsy prior to initiating treatment. As imaging and technology continue to evolve, these adjunctive techniques will likely continue to play a greater role in clinical practice.

Human Immunodeficiency Virus (HIV) Related Conjunctival Tumours

Background: The huge burden of HIV/AIDS in sub-Saharan Africa has been associated with increased prevalence of malignant conjunctival tumours especially squamous cell carcinoma (SCC). In Nigeria, the national HIV sero-prevalence is 3.4%, and values in the South-South geopolitical zone where this review was performed ranges from 4.1% in Delta to 10.9% in Akwa-Ibom state. Aim: To review HIV and conjunctival tumours in patients seen at the University of Calabar Teaching Hospital (UCTH), Calabar Methodology: This retrospective clinic review of demographic data, clinical features, HIV sero-status and histopathologic findings in patients with conjunctival tumours seen from January 2005 to December 2014 including Ophthalmology clinic records and pathology request forms were analysed Results: A total of 32,102 new patients were seen in the ophthalmic outpatient within the study period, of which 108 patients comprising 57(52.8%) males and 42(38.0%) females had conjunctival tumours. Their ages ranged from 11-85 years, with a mean of 39 years ± 14.98. Histologically, eighty (74.1%) had Squamous cell carcinoma, ten (9.26%) Squamous intraepithelial lesion, six (5.56%) Papilloma, four (3.7%) Kaposis sarcoma, three (2.78%) Naevus and Melanoma each, one each (0.93%) had Conjunctival dysplasia and Lymphoma. The HIV status of only 55 (50.9%) could be ascertained having been documented in patients’ records of whom thirty-seven (67.3%) were HIV positive. Thirty four (>90%) of the HIV positive cases had squamous cell carcinoma. The other 3 patients had kaposis sarcoma, papilloma and conjunctival intraepithelial neoplasia(CIN). Conclusion: In Calabar, conjunctival squamous cell carcinoma is preponderant in HIV sero-positive patients when compared with the sero-negatives.

Clinical features and prognosis of conjunctival lymphoid hyperplasia

Background Primary conjunctival lymphoid hyperplasia exhibit atypical clinical symptoms and easy to cause misdiagnosis and mistreatment.Understanding the clinical characteristics of primary conjunctival lymphoid hyperplasia is of an important clinical significance. Objective This study was to analyze the clinical characteristics and therapeutic outcome of patients with primary conjunctival lymphoid hyperplasia. Methods The medical records of 20 patients with primary conjunctival lymphoid hyperplasia who was treated in Beijing Tongren Hospital from January 2012 to December 2014 were retrospectively analyed.The age, symptoms, therapy, prognosis, histopathological features and immonochemistry results were reviewed, and the treating approach targeting to different types of conjunctival lymphoid hyperplasia was evaluated.The fellow-up duration was 1-2 years. Results Age of the patients ranged from 27 to 83 years old.The lesions were classified as benign lymphocytic hyperplasia, atypical lymphoid hyperplasia and lymphoma.Majority of lesions located in fornix conjunctiva tissue (90%). Follicle-like appearance was seen in the benign lymphocytic hyperplasia, and pink elavation was exhibited at the bulbar conjunctiva in the atypical lymphoid hyperplasia.A diffuse salmon fish like appearance in conjunctiva was the primary feature of conjunctival lymphoma.Histopathological examination showed that conjunctival benign lymphocytic hyperplasia had follicle-like tissue in lesions, atypical lymphoid hyperplasia exhibited diffuse lymphocyte filtration, and conjunctival lymphoma appeared monocyte filtration.The 3 tpyes of lesions also could be differentiated by immunochemistry.The medicine was applied in the eyes with benign lymphocytic hyperplasia, and the combination of resection of lesions with conjunctival tissue or amniotic membrane transplantation was used for the atypical lymphoid hyperplasia or lymphoma eyes.No recurrence of the lesions was found during fellow-up duration. Conclusions The clinical manifestations of conjunctival lymphoid hyperplasia lesions are varied, and it is easy to be confused with chronic ocular surface diseases.Pathology and immunochemistry are helpful for the differential diagnosis.The treating regimen is dependent on the lesion type.Most patients have a favorable prognosis with treatment. Key words: Conjunctival diseases/pathology; Conjunctival neoplasms/diagnosis & therapy; Hyperplasia; Lymphoid tissue/pathology; Lymphoma/pathology; Retrospective studies; Humans

Nasal dissemination of a single-clone IgH-rearranged conjunctival MALT lymphoma through the nasolacrimal duct: A case report.

The aim of the present study was to report a rare case of single-clone, immunoglobulin heavy chain (IgH)-rearranged mucosa-associated lymphoid tissue (MALT) lymphoma in the conjunctiva, with nasal cavity dissemination through the nasolacrimal duct. A 24-year-old female was diagnosed with MALT lymphoma of the nasal cavity at the Department of Otolaryngology, Wan Fang Medical Center, Taipei Medical University (Tapei, Taiwan) in October 2008. A biopsy of the relapsing conjunctival lesion revealed a MALT lymphoma by pathological staining, while a single-clone, IgH-rearranged tumor lesion in the nasal cavity and conjunctiva was confirmed using continuous sinus computed tomography scans and polymerase chain reaction. Tumor lesions were negative for Helicobacter pylori and Chlamydia infection, but exhibited bilateral neck lymph node dissemination. A combination of radiation therapy (a total dosage of 46.8 Gray, in two phases covering the left lacrimal sac, nasal cavity and bilateral neck region) and topical ciprofloxacin plus steroid (0.3% ciprofloxacin 4 times a day and betamethasone eye ointment before sleep for 1 month) was provided as an effective therapeutic strategy, and no recurrence was found in the next 3 years. The nasolacrimal duct serves as a channel for conjunctival tumor spreading and is easily neglected. IgH-involved translocation in MALT lymphoma is a factor in the progression of the disease, and aggressive combination therapy is essential for a high-risk, disseminated IgH-rearranged MALT lymphoma.

Conjunctival Lymphoma--An International Multicenter Retrospective Study.

To date, the clinical features of the various subtypes of conjunctival lymphoma (CL) have not been previously evaluated in a large cohort. To characterize subtype-specific clinical features of CL and their effect on patient outcome. A retrospective multicenter study was performed. Patient data were collected from January 1, 1980, through December 31, 2010. The dates of the analysis were May 15, 2015, to August 20, 2015. The median follow-up period was 43 months. Seven eye cancer centers were involved in the study. In total, 268 patients with CL were identified, 5 of whom were excluded because of missing clinical data. Overall survival, disease-specific survival, and progression-free survival were the primary end points. Two hundred sixty-three patients with CL were included in the study. Their mean age was 61.3 years, and 55.1% (145 of 263) were female. All lymphomas were of B-cell type. The most frequent subtype was extranodal marginal zone lymphoma (EMZL) (68.4% [180 of 263]), followed by follicular lymphoma (FL) (16.3% [43 of 263]), mantle cell lymphoma (MCL) (6.8% [18 of 263]), and diffuse large B-cell lymphoma (DLBCL) (4.6% [12 of 263). Conjunctival lymphoma commonly manifested in elderly individuals (age range, 60-70 years old), with EMZL having a female predilection (57.8% [104 of 180]) and MCL having a marked male predominance (77.8% [14 of 18]). Unlike EMZL and FL, DLBCL and MCL were frequently secondary diseases (41.7% [5 of 12] and 88.9% [16 of 18], respectively), with MCL showing a frequent occurrence of stage IVE lymphoma (61.1% [11 of 18]) and bilateral manifestation (77.8% [14 of 18]). Localized disease (stage IE or IIE) was commonly treated with external beam radiation therapy (EBRT) with or without chemotherapy, while widespread lymphoma (stage IIIE or IVE) and MCL of any stage were managed with chemotherapy with or without EBRT. Diffuse large B-cell lymphoma and MCL had a poor prognosis, with 5-year disease-specific survival of 55.0% and 9.0%, respectively, in contrast to EMZL (97.0%) and FL (82.0%). Further survival predictors included age (EMZL), sex (FL), and Ann Arbor staging classification (EMZL and FL). The American Joint Committee on Cancer TNM staging showed limited prognostic usefulness, only being able to predict survival for patients with DLBCL. Conjunctival lymphoma consists of mainly 4 subtypes of B-cell non-Hodgkin lymphoma: EMZL, FL, MCL, and DLBCL. Mantle cell lymphoma is characterized by a particularly high frequency of secondary disease of stage IVE and bilateral manifestation. The histological subtype is the main outcome predictor, with MCL and DLBCL having a markedly poorer prognosis than EMZL and FL.

Primary conjunctival follicular lymphoma mimicking chronic conjunctivitis

Clinical caseThe case is presented of a 43-year-old male patient with chronic follicular conjunctivitis, negative bacterial serology, and refractory to local treatment. The incisional biopsy performed showed to be consistent with reactive lymphoid hyperplasia. A year later, a new incisional biopsy showed follicular lymphoma, with no systemic involvement, and he was treated with local radiotherapy. DiscussionWhen a chronic follicular conjunctivitis is refractory to treatment, it is essential to perform an incisional biopsy to establish the histopathological diagnosis that can range from chronic inflammation, reactive lymphoid hyperplasia to lymphoma. Follicular lymphoma is rare among conjunctival lymphomas, and the staging is indispensable for the correct therapeutic approach.

Ocular Adnexal Lymphoma: Clinical Presentation, Diagnosis, Treatment and Prognosis

Ocular adnexal lymphoma (OAL) is a rare tumor with an incidence of 0.2 per 1,00,000 individuals. However, it constitutes the most common orbital tumor especially in older population with an incidence ranging from 11% to 24%. OAL can involve conjunctiva, orbital soft tissues, eyelid, or adnexal structures such as lacrimal gland and lacrimal drainage system. The most common primary OAL is low-grade malignant extranodal marginal zone B-cell lymphoma (EMZL) of mucosa-associated lymphoid tissue (MALT) type. Though various classification and staging systems have been proposed for OAL, currently, American joint committee on cancer (AJCC) TNM (tumor node metastasis) staging is most commonly used. Most commonly, conjunctival lymphoma typically presents as ‘salmon-colored patch’ and orbital lymphoma presents as proptosis, which on imaging shows characteristic appearance of molding around the globe without any globe indentation or bone erosion. Orbital radiotherapy and systemic chemotherapy forms the mainstay of treatment. Newer treatment modalities include immunotherapy with interferon and anti-CD20 antibody rituximab that have been shown to be useful either when used alone or in combination with chemotherapy or as radio-immunotherapy.