Ocular Extranodal Marginal Zone Lymphoma - A Study of Twenty-Two Cases Presented at Bangalore, Karnataka

BACKGROUND Ocular adnexa lympho-proliferative disorders are a divergent diverse category of ocular malignancies, comprising roughly about 1 % to 2 % of Non-Hodgkin’s lymphomas (NHLs) and 8 % of extranodal lympho-proliferative disorders. The most frequent type, approximating for up to 80 % of cases that constitutes the primary is marginal zone lymphoma of mucosa associated lymphoid tissue (MALT) type. Marginal zone lymphomas which usually have an extranodal presentation are routinely diagnosed by histomorphology by the diffuse infiltration of atypical lymphoid cells with plasmacytoid appearance and presence of pathognomonic lymphoepithelial lesions. Lymphoepithelial lesions are the existence of lymphocytes in the cytoplasm of epithelial cells. Immunohistochemistry shows a consistent absence of CD5 and CD23 staining, hence considered as diagnosis of exclusion on histo-immunomorphology. METHODS This is a case-series study. Twenty-two cases of ocular extranodal marginal zone lymphoma were obtained from the archives of pathology from 2013 to 2015. The histopathology and immunohistochemistry slides were reviewed by three expert histopathologists for confirmation of diagnosis. Immunohistochemical markers mainly used were CD45, CD3, CD5, CD20, cyclin D1, CD10, Ki - 67, BCL - 2, PAX 5 and CD23. The immunohistochemical markers such as CD10, BCL - 2 and cyclin D1 IHC expression were studied in cases of ocular extranodal marginal zone lymphoma (OENMZL). Relevant clinical details were collected from the patients such age, sex and history of autoimmune condition if any. RESULTS Eighteen cases (81 %) of OENMZL belonged to the age group of more than 40 years. There was a definite male preponderance (77 %) and it was associated with autoimmune conditions such as Hashimoto’s thyroiditis (18 %) and Sjogren’s syndrome (22 %). 22 cases of OENMZL were analysed and all showed consistent immunoexpression for CD20, CD45 while were immunonegative for CD5, CD23 and cyclin D1. 6 cases (27 %) showed CD10 positivity while 20 cases showed Bcl2 positivity (90 %). CONCLUSIONS OENMZL shows positivity for CD20 and CD45 while immunonegative for CD5, CD23 and cyclin D1, and a fraction of cases can show IHC positivity for CD10 and Bcl-2. KEYWORDS OENMZL: Ocular Extranodal Marginal Zone Lymphoma, MZL: Marginal Zone Lymphoma, CD: Cluster Differentiation. NHL: Non-Hodgkin Lymphoma. OL: Ocular Lymphoma. PCR: Polymerase Chain Reaction, FISH: Fluorescent In Situ Hybridization

Two Rare Cases of Bronchus-Associated Lymphoid Tissue Lymphoma Successfully Treated with Rituximab-Bendamustine

The bronchus-associated lymphoid tissue (BALT) lymphoma is a rare subtype of extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), affecting roughly 9% of all MALT lymphomas. The underlying pathomechanism of BALT lymphomas is not completely resolved and a standard of care treatment is so far not available.

Treatments and Outcomes in Stage I Extranodal Marginal Zone Lymphoma in the United States

A considerable number of patients with extranodal marginal zone lymphoma (EMZL) are diagnosed with stage I disease. Information on treatments and survival by primary location remains limited. We extracted data from the Surveillance, Epidemiology, and End Results (SEER) database to assess treatment, primary location, and survival of patients with stage I EMZL. Results show that 7961 patients met inclusion criteria. Observation (no treatment) was the most common approach (31%) followed by radiation therapy (RT, 23%). The median overall survival (OS) was 17.3 years (95%CI 16.3 to 18.3). Shorter survival was observed in patients with stage I EMZL compared to expected survival in a cohort derived from the general U.S. population matched by sex, age, and calendar year at diagnosis. However, similar survival was observed in RT-treated patients. We identified age ≥ 60 years (SHR = 4.00, 95%CI 3.10–5.15; p < 0.001), higher grade transformation (SHR = 4.63, 95%CI 3.29–6.52; p < 0.001), and primary lung EMZL (SHR = 1.44, 95%CI 1.05–1.96; p = 0.022) as factors associated with shorter lymphoma-specific survival (LSS). Conversely, primary skin location (SHR = 0.50, 95%CI 0.33–0.77; p = 0.002) was associated with longer LSS. Our results support the use of RT as the preferred approach in localized EMZL.

Primary Hepatic Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue in a Patient with Chronic Hepatitis B Virus Infection: Case Report and Summary of the Literature

Background: The incidence of extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) is low, at 7–8% of all non-Hodgkin lymphoma cases. The most common site of MALT lymphoma occurrence is the stomach. Primary hepatic extranodal marginal zone lymphoma of MALT is classified as a type of non-gastric MALT lymphoma and is considered extremely rare, with no consensus on imaging study findings or treatment due to a limited number of reports. We herein describe a rare case of primary hepatic extranodal marginal zone lymphoma of MALT with underlying hepatitis B infection (HBV) and present useful diagnostic findings of various imaging modalities, including contrast-enhanced ultrasonography (CEUS) with Sonazoid. Case presentation: A 66-year-old woman was diagnosed as being a non-active carrier of HBV at 51 years of age at the time of total hysterectomy and bilateral adnexectomy for uterine cervical cancer. She was admitted to our hospital following the incidental detection of two focal liver lesions on computed tomography. The lesions were considered malignant based on clinical and other radiologic imaging findings. Her CEUS results of hypo-enhancement in the portal and late phases were consistent with those of previously reported cases of hepatic extranodal marginal zone lymphoma of MALT, and histological liver biopsy findings were compatible with the diagnosis. Conclusions: Primary hepatic extranodal marginal zone lymphoma of MALT is a rare condition that can appear in HBV carriers. Characteristic CEUS findings may help in disease diagnosis. Clinicians should bear primary hepatic extranodal marginal zone lymphoma of MALT in mind when encountering patients with focal liver lesions which exhibit image findings different from those of typical hepatocellular carcinoma.