Mucosa-associated lymphoid tissue (MALT) lymphoma arising in the esophagus, stomach, and lung

Abstract

A 62-year-old woman was referred to our hospital for further investigation of slow-growing gastrointestinal submucosal tumors (SMTs) and multiple lung nodules. Esophageal SMTs had been identified 6 years earlier, following which lung tumors and gastric SMTs had subsequently developed. Despite repeated endoscopic biopsies, these SMTs could not be diagnosed definitively. Moreover, we were unable to detect any serological abnormalities or radiologic findings such as lymph node swelling. Thoracoscopic excision of a lung nodule led to the definitive diagnosis of mucosaassociated lymphoid tissue (MALT) lymphoma. Cytological findings of aspiration biopsy specimens from the esophagus and stomach were compatible with that of the lung nodule. To our knowledge, this is the first case report of esophageal MALT lymphoma with lung and gastric involvement. We discuss this extremely rare disease with reference to the relevant literature.

Primary esophageal mucosa-associated lymphoid tissue lymphoma diagnosed by using stacked forceps biopsy

“…Pathological diagnosis by using endoscopic simple forceps biopsy is difficult because the esophageal MALT lymphoma is obscured by the overlying mucosa, and it may therefore appear to be an esophageal submucosal tumor. Of 13 previously reported cases, five cases were diagnosed by using EMR, three cases by using surgery, two cases by using EUS‐FNA, and only three cases by using endoscopic biopsy . Advantages of EMR include the acquisition of a sufficiently large tissue sample for diagnosis, but it also has some associated complications, including bleeding and perforation.…”

“…Of 13 previously reported cases, five cases were diagnosed by using EMR, three cases by using surgery, two cases by using EUS-FNA, and only three cases by using endoscopic biopsy. [2][3][4][5][6][7][8][9][10][11][12][13][14] Advantages of EMR include the acquisition of a sufficiently large tissue sample for diagnosis, but it also has some associated complications, including bleeding and perforation. Although esophageal EMR is less invasive than surgery, the average rate of bleeding is 10% and the reported perforation rate is 0.3-0.5%.…”

“…26 Gastric MALT lymphoma without the t(11; 18) chromosomal translocation has been shown to regress completely after H. pylori eradication therapy 27 ; however, no standard treatment has been established for esophageal MALT lymphoma. The treatment used in 13 previously reported cases was EMR alone in two cases, 6,13 EMR followed by radiotherapy in one case, 11 surgical resection alone in three cases, 8,9,14 chemotherapy alone in three cases, 2,4,12 H. pylori eradication therapy followed by chemotherapy in one case, 10 and radiotherapy alone in two cases. 5,7 Our patient was treated with CVP chemotherapy owing to the possibility of aortocaval and iliac lymph node involvement in abdomen and pelvis.…”

“…Within the gastrointestinal tract, the stomach is the most common site of MALT lymphoma, and the esophagus is the least common site, accounting for only approximately 1% of gastrointestinal occurrences . Many esophageal MALT lymphoma cases were diagnosed by using surgical biopsy, endoscopic mucosal resection (EMR), and endoscopic ultrasound‐guided fine‐needle aspiration (EUS‐FNA) . Pathological diagnosis by using endoscopic forceps biopsy is difficult because esophageal MALT lymphoma is obscured by the overlying mucosa, and therefore, it appears similar to an esophageal submucosal tumor.…”

“…1 Many esophageal MALT lymphoma cases were diagnosed by using surgical biopsy, endoscopic mucosal resection (EMR), and endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA). [2][3][4][5][6][7][8][9][10][11][12][13][14] Pathological diagnosis by using endoscopic forceps biopsy is difficult because esophageal MALT lymphoma is obscured by the overlying mucosa, and therefore, it appears similar to an esophageal submucosal tumor. We have used a stacked forceps biopsy procedure that allows tissue samples to be successfully obtained in these circumstances, 15,16 and herein we report on its use in a case of MALT lymphoma of the esophagus.…”