Conjunctival Marginal Zone Research Articles

IBCL-342: Conjunctival Marginal Zone B-cell Lymphoma: A Descriptive Series from a Mexican Cancer Center

Context: Approximately 5-15% of extranodal lymphomas are found in the eye, frequently with conjunctival involvement. The most common histotype is MALT in 80% of cases. Few have been described regarding clinical characteristics, treatment, response and survival especially in our population. Objective: To describe the epidemiology, clinical characteristics, treatment response and survival of CALT patients in a Mexican Cancer Center. Design: A retrospective observational study was performed. Setting: A single cancer reference center (Instituto Nacional de Cancerologia) between 2008 - 2019. Patients: 58 patients with CMZL were identified. Main outcome measures: Descriptive statistics were used. OS and EFS were assessed with Kaplan Mayer curves. Results: 58 patients were included in a period of 11 years with a median follow-up of 48 (3-136) months. The median age was 57.5 (18-88) and 41% were over 60. Most patients were female (63.8%). At diagnosis, the predominant ECOG was Conclusions: CMZL is a common neoplasm which affects ocular adnexal. We observed a disease with indolent behavior in which a localized disease is observed in most patients. The impact on the physical performance is not significant and conservative therapy is valid.

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.

Systemic Rituximab for Conjunctival Marginal Zone (Malt) Lymphoma is not Protective for Subsequent Disease Development in the Contralateral Eye

Purpose: We report a case of a 35-year old male who presented with unilateral conjunctival extranodal marginal zone (MALT) lymphoma and underwent systemic Rituximab immunotherapy as primary treatment. After an excellent initial response and four years of complete remission he presents with MALT lymphoma to the contralateral conjunctiva. Methods: MALT lymphoma is the most common lymphoid neoplasm of the conjunctiva, and has an indolent clinical course. Rare cases of spontaneous remission, bilateral involvement, systemic dissemination, and common local and contralateral relapses have been reported. Rituximab is a chimeric anti-CD20 antibody, currently used as first-line treatment of CD20 positive non-Hodgkin’s lymphoma. Several mechanisms can be responsible for rituximab resistance. Transformation of CD20 positive indolent to aggressive CD20 negative form is one of them. Results: In this case, the patient did not demonstrate any systemic or orbital involvement. Both histological reports of the incisional biopsies revealed CD20 positive immunohistochemistry. Conclusion: This is a highly unusual case of new primary conjunctival MALT lymphoma to the contralateral eye after systemic Rituximab treatment. This further supports the assumption that rituximab does not suppress MALT lymphoma formation in the long term in the other eye, suggesting that the predisposing immune factors are not annihilated by the course of rituximab. Long term review of these patients is therefore warranted.

Marginal zone B-cell lymphoma of the conjunctiva

Lymphomas are the most common orbital malignancies, marginal zone B-cell lymphoma being the most common histotype. One quarter of these lymphomas exhibit conjunctival involvement, which is almost exclusively observed in indolent lymphomas (96% of conjunctival lymphomas). Primary conjunctival marginal zone B-cell lymphoma exhibits interesting and peculiar features, with some molecular, biological, clinical and prognostic differences from the rest of the ocular adnexal lymphomas. Recent studies demonstrate that chromosomal abnormalities, autoimmune disorders and infectious agents are variably associated with the development and behavior of this lymphoma. Conjunctival lymphomas are rarely lethal tumors and, in selected cases, may be managed without anti-tumor treatment. In other cases, conjunctival lymphomas cause symptoms that require immediate treatment. To date, no guidelines for the management of patients with conjunctival lymphoma exist. Surgical resection, radiotherapy and chemotherapy are the most common approaches to these malignancies, but the use of systemic immunotherapy, bacteria-eradicating antibiotic therapy and local treatments, such as intralesional injections of interferon or rituximab and eyedrops of cytostatics, is progressively growing.

Conjunctival Marginal Zone B-cell Lymphoma (MALT Lymphoma) with Amyloid and Relapse in the Stomach

The authors report a localized (primary) conjunctival marginal zone B-cell lymphoma (mucosa-associated lymphoid tissue (MALT)-type), with amyloid deposition with relapse in the stomach, 14 months after the initial diagnosis. Ocular adnexal marginal zone B-cell MALT lymphoma is often localized at diagnosis; some relapse in typical MALT sites. There are few reports of localized conjunctival lymphoma with a relapse in the stomach. The authors suggest that all patients with localized ocular adnexal lymphoma be followed for an extended period.