Encyclopedia of Pathology

Living Edition
| Editors: J.H.J.M. van Krieken

Nodal Marginal Zone Lymphoma

  • Michiel van den BrandEmail author
Living reference work entry
DOI: https://doi.org/10.1007/978-3-319-28845-1_3869-1



Nodal marginal zone lymphoma (NMZL) is a rare type of B-cell lymphoma. It is defined as a nodal B-cell neoplasm that resembles lymph nodes involved by splenic marginal zone lymphoma (SMZL) or extranodal marginal zone lymphoma (EMZL) but without evidence of splenic or extranodal disease.

Clinical Features

  • Incidence

    NMZL is a rare type of B-cell lymphoma, accounting for less than 2% of lymphoid neoplasms. However, the incidence of NMZL appears to be increasing (Olszewski and Castillo 2012).

  • Age

    The median age of presentation is around 60 years, but there is a wide variation with an age range from 13 to 92 years. Especially in younger patients, the possibility of the pediatric form of marginal zone lymphoma should be considered.

  • Sex

    Males and females are affected at approximately equal frequency.

  • Site

    By definition, the disease presents in the lymph nodes with the absence of extranodal or splenic disease. Most...

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References and Further Reading

  1. Olszewski, A. J., & Castillo, J. J. (2012). Survival of patients with marginal zone lymphoma: Analysis of the Surveillance, Epidemiology, and End Results database. Cancer, 119, 629–638.CrossRefGoogle Scholar
  2. Salama, M. E., Lossos, I. S., Warnke, R. A., & Natkunam, Y. (2009). Immunoarchitectural patterns in nodal marginal zone B-cell lymphoma: A study of 51 cases. American Journal of Clinical Pathology, 132, 39–49.CrossRefGoogle Scholar
  3. Spina, V., Khiabanian, H., Messina, M., Monti, S., Cascione, L., Bruscaggin, A., Spaccarotella, E., Holmes, A. B., Arcaini, L., Lucioni, M., Tabbo, F., Zairis, S., Diop, F., Cerri, M., Chiaretti, S., Marasca, R., Ponzoni, M., Deaglio, S., Ramponi, A., Tiacci, E., Pasqualucci, L., Paulli, M., Falini, B., Inghirami, G., Bertoni, F., Foa, R., Rabadan, R., Gaidano, G., & Rossi, D. (2016). The genetics of nodal marginal zone lymphoma. Blood, 128, 1362–1373.CrossRefGoogle Scholar
  4. Van den Brand, M., & Van Krieken, J. H. J. M. (2013). Recognizing nodal marginal zone lymphoma: Recent advances and pitfalls. A systematic review. Haematologica, 98, 1003–1013.CrossRefGoogle Scholar
  5. Van den Brand, M., Rijntjes, J., Hebeda, K. M., Menting, L., Bregitha, C. V., Stevens, W. B. C., Van der Velden, W. J. F. M., Tops, B. B. J., Van Krieken, J. H. J. M., & Groenen, P. J. T. A. (2017). Recurrent mutations in genes involved in NF-kappaB signaling in nodal marginal zone lymphoma. Histopathology, 70, 174–184.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of PathologyRadboud University Medical CenterNijmegenThe Netherlands
  2. 2.Pathology-DNARijnstate HospitalArnhemThe Netherlands