Appendiceal MALT Lymphoma in Childhood – Presentation and Evolution

Lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) was first described by Isaacson et al. in 1983 (Isaacson & Wright, 1984). According to the WHO lymphoma classification, the indolent B cell lymphoma of MALT type is classified as a marginal zone lymphoma, thus called because it originates from the B lymphocytes normally present in a distinct anatomical location (marginal zone) of the secondary lymphoid follicles (Harris et al., 2001). MALT lymphomas comprise up to 40% of adult non-Hodgkin lymphomas (NHL); the median age at occurrence is 60 years, with a female predominance (Anonymous, 1997). In paediatric age MALT lymphomas are very rare. We report on a case of MALT lymphoma involving the appendix in a 6-year-old immunocompetent girl and its evolution toward an inflammatory bowel disease (IBD) at a middle-term follow-up.