Pathology of extra-nodal non Hodgkin lymphomas

In the management of extra-nodal lymphomas it is important to determine whether the tumour has disseminated and whether lymph nodes are involved. Some extra-nodal lymphomas may be the result of random spread of nodal lymphoma. Specific homing, however, determines the site of many extra-nodal lymphomas, as exemplified by cutaneous T-cell lymphomas, which seem to be derived from skin-homing T-cells and mucosa-associated lymphoid tissue lymphomas that show features of the mucosal immune system. Enteropathy-associated T-cell lymphoma is derived from mucosal T-cells in patients with coeliac disease. Immunological sanctuary accounts for the localisation of primary brain, eye and testicular lymphoma. Mantle cell lymphoma frequently causes tumours in the gastrointestinal tract. Random biopsies have shown that a high proportion of patients with this lymphoma have extensive occult involvement of the gastrointestinal tract at the time of first diagnosis. Follicular lymphoma occurs at both nodal and extra-nodal sites, but uncommonly at both sites at the same time. Extra-nodal follicular lymphomas frequently lack t(14;18)(q32;q21) and do not express bcl-2, which are characteristics of the nodal disease. At extra-nodal sites, follicular lymphoma is more likely to be curable than nodal follicular lymphoma. The behaviour of extra-nodal lymphomas cannot be assumed to follow that of their nodal counterparts.