Nasopharyngeal B Cell Lymphoma With Both IGH/CCND1 Rearrangement and MALT1 Gene Amplification

Document Type

Conference Proceeding

Publication Date

9-2019

Publication Title

Archives of Pathology and Laboratory Medicine

Abstract

Mantle cell lymphoma (MCL) is characterized by the t(11;14)(q13;q32)/CCND1þ. MALT1 amplification is the most common genetic event in MALT lymphoma. Identification of CCND1 and MALT1 gene overexpression has a key role in the diagnosis of MCL and MALT lymphoma. Several unusual variants of mantle cell lymphoma have been described, with variable morphologic, immunophenotypic, and genetic characteristics. Here, we report an unusual nasopharyngeal B cell lymphoma with both CCND1 rearrangement and MALT1 amplification. The patient was a 60-year-old man with a recent clinical history of ‘‘cutaneous MALT lymphoma.’’ A positron emission tomography oncology study revealed intense fluorodeoxyglucose avidity in the nasopharyngeal region, highly suspicious for malignancy. A biopsy of nasopharyngeal lesion was performed. Histologic examination showed focal expansion of the mantle zone surrounding residual germinal centers. Flow cytometry demonstrated monoclonal B cells, CD23 with variable CD5 expression. Lymphocytes in the mantle zone were positive for CD20, BCL1, and weakly positive for CD5 by immunohistochemistry. Interestingly, fluorescence in situ hybridization studies were positive for standard and variant IGH/CCND1 rearrangement (85%) and MALT1 gene amplification (60%). Further staging evaluations showed minimal bone marrow lymphoma involvement and increased fluorodeoxyglucose avidity in bilateral tonsillar regions and regional nodes of the neck, indicative of systemic disease. The overall findings were consistent with nasopharyngeal mantle cell lymphoma with both CCND1 and MALT1 gene overexpression. To our knowledge, this is the first such case reported in the literature. Recent studies have shown that the MALT1 gene may be involved in the MYC pathway regulation in MCL, which represents a promising target for future therapies in patients with MCL.

Volume

143

Issue

9

First Page

e63

Comments

College of American Pathologists Annual Meeting, September 21-25, 2019, Orlando, FL

Last Page

e63

DOI

10.5858/arpa.2019-0901-AB

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