MALT lymphoma in children: A report from the NHL‐BFM study group

Abstract

Marginal zone lymphomas of MALT type comprise a considerable group of indolent B-cell non-Hodgkin lymphoma (NHL) in adult patients. In childhood, however, these tumors are extremely rare, as nearly all pediatric patients have aggressive NHL. Among 2,703 children and adolescents registered into the prospective multicenter NHL-BFM treatment studies since 1986, only 4 patients (0.1%) displayed features of MALT lymphoma. These tumors were localized in the stomach, breast, lower lid, and conjunctiva, respectively and they were associated with H. pylori infection in two patients. All children are alive but long-term follow-up will be mandatory to assess the behavior of MALT lymphoma in this age group.

Pediatric Gastric Lymphoma

“…[7][8][9][10][11][12] We experienced a case of primary gastric high-grade Burkitt-like lymphoma in a previously healthy 13-yearold boy referred for suspicion of gastritis. The clinicopathologic features of this rare entity are discussed with emphasis on diagnosis and treatment.…”

Pediatric non-Hodgkin lymphoma

“…For example, MALT lymphoma in pediatric patients usually presents as localized disease and is associated with Helicobacter pylori infection. Pediatric patients with this type of disease often require no more than local therapy consisting of surgery with or without radiation for cure [ 47 ]. Mature T-cell and NK-cell NHL are much less common in children than in adults but appear to be very aggressive, and children do just as poorly as adults with these disease types.…”

Rare Pediatric Non-Hodgkin Lymphoma

“…A clonal evolution within the B-cell population in response to the infectious process is hypothesized to lead an indolent MALT lymphoma [42]. The long evolution is believed to be a reason for the rarity of MALT lymphomas in children [43]. MALTs may be due to longstanding chronic infections with H. pylori in stomach lymphomas, Borrelia burgdorferi in skin lymphomas, HIV infection, or autoimmune diseases [42].…”

“…In a cohort of 2,703 patients with MALT lymphomas, four children were identified. The management of these patients ranged from local resection to cryotherapy, antibiotic therapy for H. pylori infection, and chemotherapy (CHOP) with radiotherapy [43]. MALT lymphomas have been reported to occur in the oral cavity, including the salivary glands and lip, and also as a primary cutaneous lymphoma [44,45].…”