A case of enteropathy-associated T-cell lymphoma (Type I) arising in stomach without refractory celiac disease

Liang Wang,Yuan Miao,Juan-Han Yu,En-Hua Wang,Xu-Yong Lin,Xue-Shan Qiu,Yang Liu

doi:10.1186/1746-1596-7-172

Abstract

Enteropathy-associated T-cell lymphoma (EATL) is a rare peripheral T-cell lymphoma which was classified into 2 types based on histology. EATL is often, but not always, associated with celiac disease. EATL type I is a large cell lymphoma which is more common in frequency and highly associated with celiac disease compared with type II. Jejunum and ileum are the common sites, although EATL can rarely occur in the duodenum, stomach and colon or outside the gastrointestinal tract. We herein presented one case of gastric EATL, which happened in a 73-year-old Chinese male patient. Histologically, the tumor was composed of polymorphic (pleomorphic, anaplastic, immunoblastic) lymphoid cells and numerous inflammatory cells, including histiocytes, neutrophils and eosnophils in the background. The pleomorphic lymphoid cells were diffuse and strongly positive for CD3 and partially positive for CD30, while negative for CD4, CD5, CD8 or CD56. The gastric EATL should be distinguished from other gastric lesions, such as peptic ulcer, poorly-differentiated adenocarcinoma and other types of lymphoma.Virtual slidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1174320824810970

Highlights

Enteropathy-associated T cell lymphoma (EATL) is a rare primary extranodal T cell lymphoma which was thought to arise from the intraepithelial cells of the small intestines [1]
EATL seldom occurs in stomach, it should be considered in the differential diagnosis when the lesion exhibits neoplastic lymphoid cells
EATL is a rare type of peripheral T-cell lymphomas in gastrointestinal tract and seldom occurs in stomach

Summary

Background

Enteropathy-associated T cell lymphoma (EATL) is a rare primary extranodal T cell lymphoma which was thought to arise from the intraepithelial cells of the small intestines [1]. EATL type I is usually associated with refractory celiac disease and covers 80-90% of all EATL cases. This type frequently has large-cell or pleomorphic cytology and seldom expresses CD8 and CD56. EATL type II is sporadic, seldom associated with celiac disease, and covers 10-20% of all EATL cases and is characterized by monomorphic cytology with frequent expression of CD8 and CD56 [2]. Microscopic features The tumor formed an ulcerating mucosal mass that invades the wall of stomach (Figure 1A-F). Immunohistochemistry The immunohistochemical study showed that the pleomorphic lymphoid cells were negative for AE1/AE3, CD5, CD8, CD56 (Figure 2A-D) and CD4, strongly positive for CD3 (Figure 2E). CD20 and Pax-5 labeled the scattered B cells amidst the inflammatory background (Figure 2G and H).

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