A case of primary esophageal mucosa-associated lymphoid tissue lymphoma with a numerical abnormality of 18q21 detected by fluorescence in situ hybridization

Abstract

Dear Editor, Mucosa-associated lymphoid tissue (MALT) lymphoma, which was first described in 1983. MALT lymphoma can develop from organs where lymphocytes are normally absent by the acquisition of MALT. The acquired MALT appears in association with chronic inflammation induced by persistent infection or autoimmune disorders such as Helicobacter pylori (H. pylori) infection, Sjogren’s syndrome, or Hashimoto’s disease. Although MALT lymphoma arises from any different organs, esophagus is a rare site of origin in the literature. We report here a primary esophageal MALT lymphoma with a numerical cytogenetic abnormality detected by fluorescence in situ hybridization (FISH). A 70-year-old woman has received endoscopy and submucosal tumors (SMT) were detected incidentally in October 2004. The examination revealed two SMT lesions (0.6×0.4 cm, 2.0×0.8 cm) approximately 26 and 28 cm from the incisor teeth (Fig. 1a). Histological examination of biopsied samples revealed that smallto medium-sized cells resembling marginal zone cells (Fig. 1b). Tumor cells were positive for CD20, CD79a, bcl-2, and negative for CD5, CD10, and cyclin D1. She had no episode of any autoimmune diseases or H. pylori gastritis. Computed tomography demonstrated no evidence of enlarged regional lymph nodes in the neck, chest, or abdomen. No bone marrow involvement was detected. We diagnosed her with primary esophageal MALT lymphoma. Endoscopic mucosal resection was performed in January 2005. Immunophenotypical analysis showed a kappa light chain restriction. FISH analysis was carried out using paraffin-embedded tissue with two differentially labeled probes each placed on upstream and downstream to MALT1, resulting three non-split signals (Fig. 1c). She received additional radiation therapy (30 Gy) after endoscopic mucosal resection. The patient is alive without any symptoms or recurrence of MALT lymphoma. MALT lymphoma exemplifies the close relationship between chronic inflammation and lymphomagenesis. It is well known that the most frequent primary involved site is the gastrointestinal tract. The lymphoma cells are preceded by the acquisition of MALT as a result of H. pylori infection. Eradication of H. pylori can result in regression of the lymphoma in 75% cases. However, additional genetic environmental or other factors should play a role, since most patients with H. pylori gastritis do not develop lymphoma. Recently cytogenetic data on MALT lymphoma has been accumulated. The t(11;18)(q21;q21) results in a Ann Hematol (2009) 88:703–704 DOI 10.1007/s00277-008-0653-y