Synonyms
Angiocentric immunoproliferative lesion; Angiocentric T-cell lymphoma; Lethal granuloma; Lethal midline granuloma; Midline malignant reticulosis; Polymorphic reticulosis
Definition
EBV-positive NK or cytotoxic T-cell lymphoma occurring in the extranodal site. Upper aerodigestive tract (nasal cavity, nasopharynx, paranasal sinuses, palate) with the nasal cavity is the prototypic site of involvement. Necrosis of tumor tissue with angiodestructive and angiocentric infiltration of tumor cells is characteristic. EBV is positive in virtually all neoplastic cells. At initial presentation, bone marrow involvement is uncommon.
Clinical Features
Incidence
The prevalence of extranodal NK/T-cell lymphoma (ENKTL), nasal-type, varies depending on ethnicity and geographic location. ENKTL accounts for 2.6–12% of NHL cases in East Asian countries and for 0.5–7.8% in Latin American countries, but only less than 1% of Caucasian cases. In East Asia and Latin America, ENKTL comprises a major...
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References and Further Reading
Chuang, S. S., Chen, S. W., Chang, S. T., & Kuo, Y. T. (2017). Lymphoma in Taiwan: Review of 1347 neoplasms from a single institution according to the 2016 revision of the World Health Organization classification. Journal of the Formosan Medical Association, 116, 620–625.
Kim, J. M., Ko, Y. H., Lee, S. S., Huh, J., Kang, C. S., Kim, C. W., et al. (2011). WHO classification of malignant lymphomas in Korea: Report of the third nationwide study. Korean Juornal of Pathology, 45, 254–260.
Laurini, J. A., Perry, A. M., Boilesen, E., Diebold, J., Maclennan, K. A., & Müller-Hermelink, H. K. (2012). Classification of non-Hodgkin lymphoma in Central and South America: A review of 1028 cases. Blood, 120, 4795–4801.
Sun, J., Yang, Q., Lu, Z., He, M., Gao, L., Zhu, M., et al. (2012). Distribution of lymphoid neoplasms in China: Analysis of 4,638 cases according to the World Health Organization classification. American Journal of Clinical Pathology, 138, 429–434.
The World Health Organization classification of malignant lymphomas in Japan: Incidence of recently recognized entities. Lymphoma Study Group of Japanese Pathologists. (2000). Pathology International, 50, 696–702.
Yang, Q. P., Zhang, W. Y., Yu, J. B., Zhao, S., Xu, H., Wang, W. Y., et al. (2011). Subtype distribution of lymphomas in Southwest China: Analysis of 6,382 cases using WHO classification in a single institution. Diagnostic Pathology, 6, 77.
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Ko, YH. (2020). Extranodal NK/T Cell Lymphoma, Nasal Type. In: Molina, T.J. (eds) Hematopathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-95309-0_3872
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DOI: https://doi.org/10.1007/978-3-319-95309-0_3872
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