Mantle Cell Lymphoma : Small Bowel

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Image Description

Mantle cell lymphoma (MCL) is an aggressive B-cell lymphoma that makes up about 2.5% of non-Hodgkin lymphomas in the US. It usually presents in the 5th and 6th decades of life at advanced stages with generalized lymphadenopathy and bone marrow involvement. Extranodal sites are frequently involved, including liver, spleen, gastrointestinal tract, Waldeyer's ring, lung, and pleura.

Gastrointestinal tract is involved in 10% to 25% of cases. It may take the form of multiple small polyps in small and large bowel (lymphomatous polyposis), a single bulky tumor nodule, superficial ulcers, or just diffuse thickening of the mucosa. Besides MCL, lymphomatous polyposis type presentation can also be caused by follicular lymphomas and marginal zone lymphomas of the MALT type. The presenting symptoms include abdominal pain, diarrhea, and melena.

This specimen photograph shows a single bulky 6.0 cm mass involving the duodenum. The patient presented with severe abdominal pain and vomiting. The histologic and immunohistochemical features were classic for mantle cell lymphoma. See cut surface in the next image.

About the Disease

Introduction: Mantle cell lymphoma (MCL) is an aggressive B-cell lymphoma that makes up about 2.5% of non-Hodgkin lymphomas in the US. It usually presents at advanced stages in the 5th and 6th decades of life with generalized lymphadenopathy and bone marrow involvement. There may also be involvement of peripheral blood, spleen, liver, gastrointestinal tract (lymphomatoid polyposis), Waldeyer's ring, lung, and pleura. Morphology: MCL consists of a uniform population of small to medium sized lymphoid cells resembling centrocytes with irregular, indented or clefted nuclei. The cells have condensed nuclear chromatin, inconspicuous nucleoli, and a thin rim of cytoplasm. The growth pattern can be nodular, diffuse, mantle zone or follicular. The blastoid variant contains larger cells with dispersed nuclear chromatin, nucleoli, and increased mitotic activity. Immunophenotype: MCL expresses CD19, CD20, CD43, surface Ig (usually IgM and IgD) with light chain restriction, FMC-7, and bcl-2. Cyclin D1 is strongly positive. It is CD5+ and CD23- which helps to separate it from CLL/SLL. CD5 expression is sometimes weak or absent in MCL. CD10 and bcl-6 are usually negative. Genetics: Most cases show t(11;14)(q13;q32) involving the IGH locus on chromosome 14 and CCND1 (cyclin D1) locus on chromosome 11 leading to overexpression of cyclin D1 which promotes cell proliferation. The translocation can be detected by karyotyping in about 70% of cases and by FISH in almost 100% of cases. The blastoid variant may show TP53 mutations, MYC translocation t(8;14)(q24;q32), and tetraploidy. These are associated with aggressive behavior. Treatment & Prognosis: MCL has poor prognosis. The median survival is 3 to 4 years. High mitotic rate (>10 mitoses/15HPF) and high Ki-67 proliferation index (>40%-60%) are associated with adverse outcome. Reference:Jaffe, E. S. et al (2017). Hematopathology - Second Edition. Philadelphia, PA. Elsevier.Jaffe, E. S. et al (2017). Hematopathology - Second Edition. Philadelphia, PA. Elsevier.

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