Thymic extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue: A clinicopathological and genetic analysis of six cases
ABSTRACT We investigated six new cases of primary thymic extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) to further characterize the clinicopathological and genetic features. The male to female ratio was 1:1. One female patient had suffered from systemic lupus erythematosus. Another female patient was diagnosed with rheumatoid arthritis after thymectomy. One patient had a concurrent tumor in the eyelid. Radiologically, all tumors were located in the prevascular space and presented as asymmetric heterogeneously enhanced cystic and solid masses. MALT1, BCL10 or IGH translocations and trisomy 18 were not observed in any cases by fluorescence in situ hybridization (FISH) analysis. Trisomy 3 was detected in one patient and another showed a TNFAIP3/A20 deletion. Meta-analysis (n = 51) including the present and previously reported cases revealed that the prevalence of autoimmunity was much lower in males with thymic MALT lymphoma compared to females (33% vs. 87%, p = 0.001). Additionally, the average age of females or patients with autoimmunity was about 10 years younger than that of males or patients without autoimmunity (p = 0.003 and p = 0.008, respectively). In summary, thymic MALT lymphoma arising without underlying autoimmunity frequently involves males or older patients. Trisomy 3 and an A20 deletion might play a role in the pathogenesis of thymic MALT lymphoma.
- 09/2011; 4(3):135-144. DOI:10.1007/s12308-011-0112-x
- [Show abstract] [Hide abstract]
ABSTRACT: INTRODUCTION: Thymic mucosa-associated lymphoid tissue (MALT) lymphoma involving lymph nodes is quite rare with only 13 previous cases reported in the literature. PRESENTATION OF CASE: The 33-years-old female was referred to our department for the investigation of abnormalities on computed tomographic (CT) scans. CT scans showed a 9-cm×3-cm mass composed of a mixture of soft tissue and fat at the anterior mediastinum with lymphadenopathy in the neck, axillary and mediastinal regions. She was underwent complete surgical resection of the mass with regional lymph node dissection through a median sternotomy. Histological examination of the surgical specimens confirmed the diagnosis of MALT lymphoma arising in the thymus with nodal metastasis. She achieved complete remission after postoperative rituximab combined chemotherapy. DISCUSSION: Thymic MALT lymphoma occurs most frequently in Asian female aged 40-60 years and commonly appears anterior mediastinal masses on CT scans. The excised tissue is necessary to confirm the accurate histological diagnosis. The disease usually remains localized for a long time, making local surgical resection highly effective. However, when the lymph nodes are involved, effective treatment approaches of the disease is still undefined. CONCLUSION: We report a case of thymic MALT lymphoma involving lymph nodes, in which the patient was successfully treated with primary site resection with regional lymph node dissection followed by rituximab combined chemotherapy. Surgery provided not only a useful approach for collecting tissue for an accurate histological diagnosis, but also an effective local treatment, even in the case of advanced-stage thymic MALT lymphoma.International Journal of Surgery Case Reports 12/2012; 4(3):250-252. DOI:10.1016/j.ijscr.2012.12.002
- [Show abstract] [Hide abstract]
ABSTRACT: To compare clinicopathologic and molecular characteristics of low-grade gastric mucosa-associated lymphoid tissue lymphoma depending on Helicobacter pylori positivity and to find out a predictive factor for unresponsiveness to Helicobacter pylori eradication therapy in Korea. A total of 53 Helicobacter pylori-positive and 13 negative mucosa-associated lymphoid tissue lymphoma patients were enrolled, and tissues from 21 patients were investigated to examine the presence of t(11;18)(q21;q21) with fluorescence in situ hybridization. Clinicopathologic features such as the endoscopic appearance, dominant site of lesion, depth of invasion, clinical stage, and the existence of MALT1 gene rearrangement were compared between these two groups. Fifty-six patients who underwent H. pylori eradication therapy were divided into responder and nonresponder groups. The two groups were analyzed to calculate odds ratios for resistance to the eradication. Helicobacter pylori-negative gastric mucosa-associated lymphoid tissue lymphoma patients averaged a more advanced clinical stage than H. pylori-positive (p = .023) patients. The frequency of t(11;18)/API2-MALT1 did not differ between H. pylori-positive (45.5%) and H. pylori-negative cases (55.6%). Thirty-eight of 51 (74.5%) H. pylori-positive patients achieved complete regression after the eradication, while 2 of 5 (40%) H. pylori-negative patients obtained regression. Presence of lesions in both distal and proximal parts of stomach (p = .041) and bearing of t(11;18)(q21;q21) (p = .007) were predictors for nonresponsiveness for H. pylori eradication. Helicobacter pylori eradication could be performed as a primary therapy regardless of H. pylori status, and assessing t(11;18)/API2-MALT1 would be considered after failure to remission by H. pylori eradication.Helicobacter 01/2013; 18(3). DOI:10.1111/hel.12033 · 2.99 Impact Factor