Steady advances in our scientific understanding of primary extranodal mucos
a-associated lymphoid tissue-type lymphomas have been achieved during the p
ast 14 years, Leading to their inclusion within the International Lymphoma
Study Group (revised European-American classification of lymphoid neoplasms
, known as REAL) classification as extranodal B-marginal zone lymphomas. Al
though most cases conform to prototype criteria, exceptional cases, such as
those discussed at the Fourth Slide Workshop of the Society for Hematopath
ology on Extranodal Hematopoietic/Lymphoid Disorders, September 11-13, 1997
raise issues relating to microscopic, immunophenotypic, and clinical behav
ioral limits for diagnostic criteria. Furthermore, questions about therapeu
tic management have only begun to be addressed.