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Primary Extranodal Lymphomas of the GI Tract, Lung, CNS, and Skin with Common Mimics

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Practical Lymph Node and Bone Marrow Pathology

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Abstract

Lymphomas that arise at extranodal sites comprise about a fourth to third of all lymphomas. The types of lymphoma vary from one extranodal site to another and also differ from those seen in lymph nodes. Many types of extranodal lymphomas are associated with underlying autoimmune disease, infection, or immunodeficiency syndrome. For example, in the gastrointestinal (GI) tract, Helicobacter pylori infection or celiac disease predisposes an individual to the development of certain types of lymphomas. Different chromosomal translocations can be associated with lymphomas of the same histologic type at different sites. For example, the t(11;18) (q21;q21) translocation is more commonly found in marginal zone lymphomas arising in the lung and stomach than those occurring in other sites. The disease progression of lymphomas of the same histologic type can differ, depending on the site of origin. For example, primary central nervous system diffuse large B-cell lymphoma (DLBCL) has much worse prognosis than DLBCL at other extranodal sites. The diagnosis of lymphomas at extranodal sites can be challenging. Many of the specimens are small biopsies with crush artifact. The lymphoid infiltrate usually lacks the architectural features seen in the lymph node. Flow cytometry studies might not be performed because lymphoma can be an unexpected finding. In addition, many inflammatory conditions at the tissue site can mimic lymphomas. Familiarity with the types of lymphomas and their mimics that occur at different extranodal sites is helpful for establishing a diagnosis. In this chapter, we will discuss the most common lymphomas and mimics at four common extranodal sites: the gastrointestinal tract, lung, CNS, and skin. Among GI tract lymphomas, gastric and intestinal lymphomas are discussed separately.

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Acknowledgments

I would like to thank Drs. Andrew Bollen, Kirk Jones, Sarah Umetsu, Yi Xie, Melike Pekmezci, Arash Eslami, and Ifeoma Perkins for providing cases.

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  1. Lab Medicine, University of California San Francisco, San Francisco, CA, USA

    Linlin Wang

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  1. Linlin Wang
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  1. Department of Pathology, Duke University School of Medicine and Duke Health System, Durham, NC, USA

    Endi Wang

  2. Department of Pathology, Duke University School of Medicine and Duke Health System, Durham, NC, USA

    Anand Shreeram Lagoo

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Wang, L. (2020). Primary Extranodal Lymphomas of the GI Tract, Lung, CNS, and Skin with Common Mimics. In: Wang, E., Lagoo, A.S. (eds) Practical Lymph Node and Bone Marrow Pathology. Practical Anatomic Pathology. Springer, Cham. https://doi.org/10.1007/978-3-030-32189-5_12

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  • DOI: https://doi.org/10.1007/978-3-030-32189-5_12

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