Extranodal NK/T-Cell Lymphoma, Nasal Type
Extranodal natural killer (NK)/T-cell lymphoma (NKTCL), nasal type, has been defined as a distinct clinicopathologic entity since 1994 (Harris et al. Blood 84:1361–1392, 1994; Chan et al. Extranodal NK/T-cell lymphoma, nasal type. In: Jaffe ES, Harris NL,
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Yexiong Li
Abstract
Extranodal natural killer (NK)/T-cell lymphoma (NKTCL), nasal type, has been defined as a distinct clinicopathologic entity since 1994 (Harris et al. Blood 84:1361–1392, 1994; Chan et al. Extranodal NK/T-cell lymphoma, nasal type. In: Jaffe ES, Harris NL, Stein H, Vardiman JW (eds) World Health Organization classification of tumours: pathology and genetics of tumours of haematopoietic and lymphoid tissues. IARC Press, Lyon. p. 204–207, 2001; Chan et al. Extranodal NK/T-cell lymphoma, nasal type. In: Jaffe ES, Harris NL, Stein H, Vardiman JW (eds). World Health Organization classification of tumours: pathology and genetics of tumours of haematopoietic and lymphoid tissues. IARC Press, Lyon. p. 285–288, 2008), with an aggressive clinical course. It is rarely diagnosed in Europe and North America, but relatively common in East Asia and South America, accounting for 10–30 % of all cases of non-Hodgkin’s lymphomas (NHL) in these countries (Vose et al. J Clin Oncol 26:4124– 4130, 2008; Au et al. Blood 113:3931–3937, 2009; Sun et al. Am J Clin Pathol 138:429–434, 2012; Yang et al. Diagn Pathol 6:77, 2011). This disease can arise within any extranodal organ or tissue, but usually involves the upper aerodigestive tract (UADT), such as the nasal cavity and Waldeyer’s ring (Aviles et al. Clin Lab Haematol 22:215–220, 2000; Li et al. Cancer 83:449–456, 1998; Li et al. J Clin Oncol 24:181–189, 2006, Li et al. Blood 112:3057–3064, 2008; Li et al. Clin Cancer Res 15:2905– 2912, 2009). Clinically, it is characterized by predominance in adult males, a large proportion of early-stage diseases, good performance status, extensive primary tumor invasiveness, low-risk score by International
Y. Li, MD Department of Radiation Oncology, Cancer Hospital and Institute, Chinese Academy of Medical Sciences (CAMS) and Peking Union Medical College (PUMC), Beijing 100021, People’s Republic of China e-mail: [email protected]; [email protected] © Springer International Publishing Switzerland 2017 B.S. Dabaja, A.K. Ng (eds.), Radiation Therapy in Hematologic Malignancies, DOI 10.1007/978-3-319-42615-0_11
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Prognostic Index (IPI), and a propensity for extranodal spread (Au et al. Blood 113:3931–3937, 2009; Aviles et al. Clin Lab Haematol 22:215– 220, 2000; Li et al. Cancer 83:449–456, 1998; Li et al. J Clin Oncol 24:181–189, 2006; Li et al. Blood 112:3057–3064, 2008; Li et al. Clin Cancer Res 15:2905–2912, 2009). The prognosis of NKTCL varied, mainly depending on the prognostic factor and treatment (Cheung et al. Int J Radiat Oncol Biol Phys 54:182– 190, 2002; Chim et al. Blood 103:216–221, 2004). The rarity and heterogeneity of NKTCL and the lack of prospective trial data have resulted in a variety of treatment options, chemotherapy regimens, and radiotherapy volumes and doses at different institutions. However, radiotherapy is the backbone of curative intent for early-stage NKTCL. New regimen chemotherapy shows promising results against refractory or advanced NKTCL.
Clinical Presentation Case 1 A 39-year
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