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(American Journal of Pathology. 2003;162:1113-1122.)
© 2003 American Society for Investigative Pathology

API2-MALT1 Fusion Defines a Distinctive Clinicopathologic Subtype in Pulmonary Extranodal Marginal Zone B-Cell Lymphoma of Mucosa-Associated Lymphoid Tissue

Mitsukuni Okabe^*, Hiroshi Inagaki^*, Koichi Ohshima, Tadashi Yoshino, Chunmei Li^*, Tadaaki Eimoto^*, Ryuzo Ueda and Shigeo Nakamura^¶

From the Department of Pathology^* and the Department of Medicine, Nagoya City University Medical School, Nagoya; the Department of Pathology, Fukuoka University Medical School, Fukuoka; the Department of Pathology, Okayama University Medical School, Okayama; and Pathology and Molecular Diagnosis, Aichi Cancer Center Hospital, Nagoya, Japan

t(11;18)(q21;q21) is associated with mucosa-associated lymphoid tissue (MALT)-type lymphoma and results in API2-MALT1 fusion. However, its clinicopathologic significance remains unclarified. API2-MALT1 fusion is detected most frequently in MALT lymphomas primarily involving the lung. We therefore screened 51 cases of pulmonary MALT lymphoma for API2-MALT1 fusion, and studied its relationship with clinicopathologic factors including the immunohistochemical expression of BCL10, another MALT lymphoma-associated molecule. The API2-MALT1 fusion transcript was detected in 21 of 51 (41%) cases, and was correlated with the absence of any underlying autoimmune disease, and with a normal serum lactate dehydrogenase, a "typical" histology without marked plasmacytic differentiation or an increased number of large cells, and aberrant nuclear BCL10 expression. However, its prognostic impact was not identified in the limited follow-up (6 to 187 months, median 27). These data suggest that the API2-MALT1 fusion may be a causative gene abnormality unrelated to autoimmune disease. In addition, this alteration may define a homogeneous MALT lymphoma subtype that is clinically more indolent and histologically more "typical." Aberrant nuclear BCL10 expression may have a possible role as a tool to screen for this API2-MALT1 fusion. A large-scale study with a long follow-up is necessary to establish the prognostic significance of API2-MALT1 fusion.

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