Extranodal NK/T-cell lymphoma, nasal type

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Extranodal NK-T-cell lymphoma
Classification and external resources
Specialty oncology
ICD-10 C86.0
ICD-O M9719/3
MeSH D054391

Extranodal NK/T-cell lymphoma, nasal type[1][2] which was known as angiocentric lymphoma in the REAL classification,[1] and also as nasal-type NK lymphoma,[3] NK/T-cell lymphoma,[3] and polymorphic/malignant midline reticulosis[3] is a cutaneous condition which in Korea is reported to be the most common form of cutaneous lymphoma after mycosis fungoides.[4]:739

Extranodal NK-T-cell lymphoma is a type of lymphoma.[5][6][7]

It is called "extranodal" to emphasize that the location is typically not in the lymph node, and is sometimes further qualified as "nasal type". The nasal cavity, nasopharynx and upper aerodigestive tract are often involved, although extranasal presentations do occur (skin, gastrointestinal tract, eye, testis, lung, soft tissue).[1] There is a strong association with Epstein–Barr virus.[1][8][9]

The NCCN guidelines recommend either high dose radiotherapy alone for stage I without high risk features,[10] or concurrent chemoradiotherapy for stage I and II with either of two regimens.[10][11][12] L-asparaginase containing regimens have been used in advanced stage disease.

See also[edit]


  1. ^ a b c d Jaffe, ES; Harris NL, Vardiman JW, Campo E, Arber, DA. (2011). Hematopathology (1st ed.). Elsevier Saunders. ISBN 9780721600406. 
  2. ^ Swerdlow, Steven H.; International Agency for Research on Cancer; World Health Organization (2008). WHO classification of tumours of haematopoietic and lymphoid tissues. World Health Organization classification of tumours 2 (4th ed.). International Agency for Research on Cancer. ISBN 9789283224310. 
  3. ^ a b c Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0. 
  4. ^ James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0. 
  5. ^ Lee J, Suh C, Park YH; et al. (February 2006). "Extranodal natural killer T-cell lymphoma, nasal-type: a prognostic model from a retrospective multicenter study". J. Clin. Oncol. 24 (4): 612–8. doi:10.1200/JCO.2005.04.1384. PMID 16380410. 
  6. ^ Lee J, Park YH, Kim WS; et al. (July 2005). "Extranodal nasal type NK/T-cell lymphoma: elucidating clinical prognostic factors for risk-based stratification of therapy". Eur. J. Cancer 41 (10): 1402–8. doi:10.1016/j.ejca.2005.03.010. PMID 15963893. 
  7. ^ Lee J, Au WY, Park MJ; et al. (December 2008). "Autologous hematopoietic stem cell transplantation in extranodal natural killer/T cell lymphoma: a multinational, multicenter, matched controlled study". Biol. Blood Marrow Transplant. 14 (12): 1356–64. doi:10.1016/j.bbmt.2008.09.014. PMID 19041057. 
  8. ^ Stadlmann S, Fend F, Moser P; et al. (November 2001). "Epstein-Barr virus-associated extranodal NK/T-cell lymphoma, nasal type of the hypopharynx, in a renal allograft recipient: case report and review of literature". Hum. Pathol. 32 (11): 1264–8. doi:10.1053/hupa.2001.28962. PMID 11727268. 
  9. ^ Ferenczi K, Summers P, Aubert P; et al. (December 2008). "A case of CD30+ nasal natural killer/T-cell lymphoma". Am J Dermatopathol 30 (6): 567–71. doi:10.1097/DAD.0b013e318184bc3f. PMID 19033930. 
  10. ^ a b add NCCN ref
  11. ^ Kim, S. J.; Kim, K; Kim, B. S.; Kim, C. Y.; Suh, C; Huh, J; Lee, S. W.; Kim, J. S.; Cho, J; Lee, G. W.; Kang, K. M.; Eom, H. S.; Pyo, H. R.; Ahn, Y. C.; Ko, Y. H.; Kim, W. S. (2009). "Phase II trial of concurrent radiation and weekly cisplatin followed by VIPD chemotherapy in newly diagnosed, stage IE to IIE, nasal, extranodal NK/T-Cell Lymphoma: Consortium for Improving Survival of Lymphoma study". Journal of Clinical Oncology 27 (35): 6027–32. doi:10.1200/JCO.2009.23.8592. PMID 19884539. 
  12. ^ Yamaguchi, M; Tobinai, K; Oguchi, M; Ishizuka, N; Kobayashi, Y; Isobe, Y; Ishizawa, K; Maseki, N; Itoh, K; Usui, N; Wasada, I; Kinoshita, T; Ohshima, K; Matsuno, Y; Terauchi, T; Nawano, S; Ishikura, S; Kagami, Y; Hotta, T; Oshimi, K (2009). "Phase I/II study of concurrent chemoradiotherapy for localized nasal natural killer/T-cell lymphoma: Japan Clinical Oncology Group Study JCOG0211". Journal of Clinical Oncology 27 (33): 5594–600. doi:10.1200/JCO.2009.23.8295. PMID 19805668.