Radiation Therapy in Mantle Cell Lymphoma

Abstract

Mantle cell lymphoma (MCL) is a definitive subtype of non-Hodgkin’s lymphoma (NHL), first coined in the early 1990s. It is unfrequent and accounts for 6–8% of all NHLs in Europe, with increasing incidence during the past decades, particularly with regard to advanced stage disease. MCL shows the poorest prognosis of all lymphomas. Involvement of extranodal sites is common, MCLs present the highest rates of secondary gastrointestinal and extranodal involvement of all lymphomas. Most patients show symptoms due to a rapidly growing lymphadenopathy. Lymphoma classification should be based on pathological investigation of affected lymph nodes and the quantification of Ki-67 expression, considered as the most established biological risk factor in MCL, should always be determined at diagnosis. No dependable markers are verified to predict an indolent future behavior of MCL. Gastrointestinal (GI) involvement in MCL patients can lead to intestinal obstruction, perforation, or bleeding and these symptoms could require immediate surgical intervention. Due to advanced spread of the disease, at initial presentation most patients require intensive systemic treatment. Most patients present after the age of 65 and are not physically fit enough to cope with aggressive chemotherapy; they are treated with moderate immunochemotherapy regimens. For young and fit patients, aggressive immunochemotherapy followed by autologous stem cell transplantation (autoSCT) and maintenance therapy might be considered. A small share of patients presents with limited stage disease at initial diagnosis; they can benefit from radiotherapy to manage local disease, followed or preceded by a shortened chemotherapy regimen. Some patients might benefit from a wait-and-see strategy, without the immediate application of systemic or local therapy. However, it remains difficult to identify those patients. Treatment of MCL in the majority of cases remains palliative with commonly aggressive course of disease, long-term survival is just seen in few cases.