Natural killer-like T-cell lymphoma in the small intestine of a child without evidence of enteropathy

A previously healthy 6-year-old boy developed symptoms of small intestinal obstruction and was found to have a large intraabdominal mass. At laparotomy the mass involved the jejunum and adjacent mesenteric lymph nodes, requiring resection. Microscopic and immunohistochemical studies demonstrated a T-cell non-Hodgkin's lymphoma, confirmed by finding clonal T-cell receptor- beta and -gamma gene rearrangements by Southern blot analysis. The immunophenotype of this lymphoma - CD3⁺CD4^-CD8^-CD56⁺TIA-1⁺βF1^- - suggests that the tumor cells are cytotoxic natural killer (NK)-like T cells, probably of CD3⁺CD4^-CD8^- intraepithelial cell origin. Examination of the adjacent and distal small intestinal mucosa failed to show any significant pathologic change. This case was unusual because intestinal lymphomas in children are usually of B-cell origin and most commonly have small noncleaved cell morphology. Childhood intestinal T-cell lymphomas have not been the focus of specific study but appear to be rare. In adults, intestinal T-cell lymphomas often arise in the background of gluten-sensitive enteropathy (celiac disease). In contrast, this child had peripheral T-cell lymphoma, with NK-like T-cell features, in the small intestine with no clinical or histologic evidence of enteropathy.