Clinical features, treatment and outcome of mucosa-associated lymphoid tissue (MALT) lymphoma of the ocular adnexa: single center experience of 60 patients (original) (raw)
Related papers
Annals of Hematology, 2005
Non-Hodgkin's lymphomas of the orbit and ocular adnexa (OOA), the majority of which are extranodal marginal zone B-cell lymphomas of mucosaassociated lymphoid tissue-type (MALT lymphomas), are a rare disorder. The aim of this study was to evaluate the clinical features and treatment outcomes and complications in patients with MALT lymphoma of OOA. Thirty-seven patients with a histologically verified diagnosis of MALT lymphoma of OOA were included in this retrospective, observational case study. There were 22 (59%) men and 15 (41%) women, with a median age of 44 years (range, 21-80 years). The most common presenting complaint was a slowly growing orbital mass. The stages were IA E in 74%, IA EE (bilateral involvement) in 18%, IIIA E in 6%, and IVA E in 3%. None of the patients had an elevated value of LHD or β 2 -microglobulin. Surgical resection alone was attempted as the sole treatment in two patients, but tumor recurred 19 and 24 months after surgery. Radiotherapy, with a median tumor dose of 3,060 cGy, was administered in 29 patients; all of the patients achieved complete remission, and none of them had severe later complications. Combination chemotherapy alone was employed in three patients with stage IIIA E or IVA E . With a median follow-up duration of 21 months, the 3-year overall survival (OS) rate and event-free survival (EFS) rate were 97% and 86%, respectively. Subgroup analysis of the patients with localized disease, who received radiotherapy as an initial treatment modality, revealed that 3-year OS rate and EFS rate were 100% and 93%, respectively. All disease recurrences were documented histologically as MALT lymphoma. In conclusion complete staging evaluation is needed to select an adequate treatment modality. Radiotherapy alone can produce excellent local control and survival in patients with localized MALT lymphoma of OOA. Systemic chemotherapy should be considered in patients with advanced stages or systemic manifestation.
Effect of Chemotherapy on Primary Mucosa-Associated Lymphoid Tissue Lymphoma of the Orbit
Journal of Oral and Maxillofacial Surgery, 2008
Purpose: To present a treatment plan for localized mucosa-associated lymphoid tissue lymphoma of the orbit in order to preserve vital structures and function from the side effects of radiotherapy. Materials and Methods: Study of 2 clinical cases by means of clinical and radiologic examination, with 2 and 3 years follow-up, respectively. Results: Immediate remission of the disease after 6 cycles of chemotherapy, with no signs of recurrence after 2 and 3 years. Clinical examination of the oculomotor mechanism and visual activity gave excellent results. Conclusion: Although radiotherapy is preferable for localized lymphoproliferative lesions, chemotherapy should also be considered as an effective treatment that preserves the integrity and function of the ocular adnexa.
Extranodal marginal zone lymphoma of the ocular adnexa
Blood, 2009
Lymphomas of the ocular adnexa are a heterogeneous group of malignancies, composing approximately 1% to 2% of non-Hodgkin lymphomas (NHLs) and 8% of extranodal lymphomas. 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. In the recent past, there have been significant advances in our understanding of the clinical characteristics, morphology and phenotype, etiology, pathogenesis, diagnosis, natural history, treatment approaches, outcome, and prognostic factors of this disease entity. Novel immunologic and molecular techniques have aided in the distinction between MALT lymphoma and other lymphoproliferative disorders and led to the identification of tissue markers of prognostic significance. Modern imaging modalities provide invaluable tools for accurate staging and treatment planning. Besides radiotherapy and chemotherapy, a variety of new treatment options have em...
Orbital MALT Lymphoma: A Case Report
A case of orbital MALT (mucous associated lymphoid tissue) lymphoma is reported for its rarity. It presented as a large tumor obscuring the whole eye with loss of vision, without any signs of dissemination and remained free of recurrence or metastasis 12 months after undergoing simple surgical excision.
Orbital lymphoma: diagnostic approach and treatment outcome
World Journal of Surgical Oncology, 2013
Background: Lymphomas of the orbit and orbital adnexae are rare tumors, comprising only 1% of all non-Hodgkin's lymphoma. The majority of non-Hodgkin's lymphomas of the orbit are extranodal marginal-zone B-cell lymphomas of mucosa-associated lymphoid tissue type. Because of nonspecific clinical signs and symptoms, some diagnostic delay may occur. The purpose of the study was to evaluate the diagnostic approach in orbital lymphomas and to analyze their treatment outcome. Methods: In the period from 2005 to 2012, from a group of 135 patients with tumors of the orbit, we identified 11 patients diagnosed with orbital lymphoma. This patient cohort was reviewed retrospectively. Results: The patient group consisted of 11 patients (seven females, male males) with a median age of 57.7 years (range 42 to 88 years). Orbital swelling, pain and motility impairment were the leading clinical symptoms. Diagnosis was confirmed by surgical biopsy. Depending on the anatomic location of the tumor, a surgical biopsy was taken using a blepharoplasty incision, a lateral orbitotomy or a navigation-guided biopsy. The predominant histology was extranodal non-Hodgkin's lymphoma of mucosa-associated lymphoid tissue type (82%). All patients underwent complete clinical staging. These were clinical stage I EA in seven patients, and stages II EA (n = 2) and III EA (n = 2) in four patients. Patients in stage I EA were treated with radiation therapy alone, with radiation doses between 25 and 40 Gy, and patients with stage II EA received systemic chemotherapy with bendamustin/rituximab. Those two patients diagnosed with diffuse large B-cell lymphoma and mantle cell lymphoma received systemic chemotherapy according to the R-CHOP protocol. Conclusions: Owing to unspecific clinical symptoms, some diagnostic delay may occur in orbital lymphoma. If unspecific orbital symptoms are present, adequate imaging studies followed by early surgical biopsy will contribute to early diagnosis. Once diagnosis is established and staging is complete, radiation therapy is the recommended treatment for stage I EA patients. Systemic chemotherapy is indicated in selected stage II EA patients and in patients with stage III EA disease.
Treatment of primary orbital lymphomas
European Journal of Haematology, 2004
Extranodal presentation occurs in about 40% of patients with non-Hodgkin's lymphoma (NHL), and the orbit accounts for 5-14% of all cases (1). According to the Revised European-American Lymphoma (REAL) classification (2), the commonest subtype of lymphoma originating in the orbit is the extranodal marginal zone lymphoma (EMZL) of B-cell origin. When encountered in relation to an epithelial surface or glandular organ, it is thought to originate from mucosa-associated lymphoid tissue (MALT) (3). MALT can arise from the conjunctiva and lacrimal gland. However, some deep orbital MZL do not appear to have a conjunctival component. The REAL classification proposed the term extranodal marginal zone B-cell lymphoma to incorporate both the mucosa-and non-mucosa-associated MALT lymphomas (2). Other indolent lymphomas represent follicular, small B-lymphocytic, and mantle cell lymphomas. The commonest aggressive type is the diffuse large B-cell lymphoma (DLCL) (1). EMZL carries a better prognosis than other histologic types. Eighty-five percent of orbital lymphoma is of small cell (indolent) histology. In a large series of 212 patients (1) the frequency of EMZL was 43%, of lymphoplasmacytic/lymphoplasmacytoid lymphoma (LPL) 23%, of follicular lymphoma (FCL) 14%, of DLCL 10%, and of other types 10%. EMZL and diffuse LPL have a similar risk of dissemination at diagnosis, but the risk is increased in patients with FCL (4). DLCL is associated with a higher risk of progression and death. Seventy-five percent of orbital lymphomas appear hyperintense to fat on T2-weighted images and become brighter relative to their appearance on T1-weighted images. Eighty-five percent of orbital inflammatory syndrome appears isointense to fat on T2-weighted images and become slightly darker or unchanged relative to their appearance on T1-weighted images (5). Tumor density and enhancement are not useful for differentiation. This report summarizes a retrospective analysis of 15 orbital lymphomas treated at the Neurosurgical Clinic of Duisburg over a 9-yr period. The
Treatments for Ocular Adnexal Lymphoma
Ophthalmology
Purpose: To review the literature to determine the efficacy of available treatments for ocular adnexal lymphoma (OAL) and to evaluate the outcomes and complications of treatments in patients older than 13 years. Methods: A literature search was conducted last in March 2017 in the PubMed and Cochrane Library databases for English-language original research investigations that evaluated treatment outcomes for OAL. The searches identified 307 unique citations, and 27 studies were selected according to the criteria outlined for this assessment. Results: The 27 studies reviewed comprised 2009 patients. Seventy-five percent of the cases reported were extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT). Twenty-five studies reported results using radiotherapy with or without chemotherapy or surgery. The local control rate of MALT lymphomas with treatments involving radiotherapy averaged 95.9%. Distant and local relapses can occur, and in studies reporting only on MALT lymphomas (884 patients), the 5-year and 10-year disease-free survival rates were reported to be 86.4% and 78.7%, respectively. However, overall survival in patients receiving radiotherapy remained very good, with the 5-year and 10-year survival rates reported to be 93.8% and 84.9%, respectively. Studies that included data on multiple histologic subtypes of lymphoma or non-MALT lymphomas (988 patients) reported local control rates to be 93.1%; 5-year and 10-year disease-free survival rates to be 75.7% and 71.0%, respectively; and 5-year and 10-year overall survival rates to be 78.9% and 73.5%, respectively. Studies on the use of doxycycline for MALT lymphomas (137 patients) reported complete responses of between 4.4% and 13%. Complete and partial responses combined were between 26.7% and 65%. Disease-free survival was not reported for these 2 studies, although progression-free survival was reported to be between 55% and 60.9%. The most frequently reported complications of treatment were cataracts (12.1%) and dry eye (8.5%). Conclusions: For MALT lymphomas, local control, disease-free survival, and overall survival are good with radiation treatment. The results of treatment of non-MALT lymphomas using radiotherapy also were good, but they were not as favorable as the treatment results of MALT lymphomas.
Ocular adnexal lymphoma---comparison of MALT lymphoma with other histological types
British Journal of Ophthalmology, 1999
Aims-To correlate histological features of ocular adnexal lymphoma using the revised European American lymphoma classification (REAL), with stage of disease at presentation, treatment modalities, and patient outcome. MALT lymphoma defines an extranodal marginal zone B cell lymphoma as outlined in the REAL classification. Comparison groups of patients included those with primary ocular adnexal MALT lymphoma versus primary ocular adnexal lymphomas of other types, MALT lymphoma versus non-MALT lymphomas (primary and secondary), and primary ocular adnexal lymphoma (MALT lymphomas and other types) versus secondary ocular adnexal lymphomas. Methods-A retrospective review of the National Ophthalmic Pathology Laboratory records identified 20 cases of ocular adnexal lymphoma over a 10 year period which were reclassified using appropriate immunohistochemical stains. Patients' medical records were examined for data including stage of the disease at presentation, mode of treatment, and patient outcome. Results-Among the 20 cases identified 14 had primary ocular adnexal lymphomas. 10 of the primary lymphomas had histological features of MALT lymphoma. One case was a primary ocular adnexal T cell lymphoma, one a follicular centre, follicular B cell lymphoma, and two were large cell B cell lymphomas. Six cases had systemic disease, four large B cell, one follicular centre, follicular B cell, and one mantle cell. A significantly higher proportion of patients with MALT lymphomas had early disease (p = 0.005), initially required local treatment (p = 0.005) and were alive at last follow up (p = 0.001) than those without. Two patients with MALT lymphoma had recurrence of lymphoma which responded to further treatment. Conclusions-Patients with primary ocular adnexal MALT lymphomas present with localised disease requiring local treatment and have a better outcome compared with patients with other types. As a small percentage of these tumours recur, patients should be followed up indefinitely.