What do we know about duodenal-type follicular lymphoma? From pathological definition to treatment options

Duodenal-type follicular lymphoma (DFL) is a newly recognised variant of follicular lymphoma (FL), although little is known about its biology and clinical evolution. In general, patients tend to have mild symptoms and do not require therapy, comparable with other forms of low-tumour burden asymptomatic FL. Specific pathological features, such as a dendritic cell meshwork, low expression of CD10 and upregulation of activation-induced cytidine deaminase can help the diagnosis. The molecular landscape of DFL is similar to the classical nodal presentation of FL, although studies using gene expression profiling demonstrate a close relation with MALT lymphomas. Markers associated with inflammation have suggested that the microenvironment plays a likely role in the pathogenesis of DFL and its low progression rate. Clinical series published vary between 20-63 patients with an estimated overall survival between 92-100% and a median follow-up ranging between 20 and 107 months. Treatment options include a watch and wait strategy, rituximab monotherapy and radiotherapy. In this review, we summarise current pathological data and treatment studies in DFL.