1.Advances of treatment for extranodal NK/T-cell lymphoma --- review.
Song-Song ZHANG ; Min WEI ; Li YU
Journal of Experimental Hematology 2011;19(4):1075-1078
Extranodal NK/T-cell lymphoma is a rare pathological type, incidence of which is 2% - 10% of the primary NHL cases, showing a broad morphologic spectrum with frequent necrosis. Extranodal NK/T-cell lymphoma occurs more frequently in Asian population, especially in Southern China and Southeast Asian population. It is reported that the incidence of ENKL among lymphomas (1314 cases) in Asian countries was 4 times as much as the Western countries (22% vs. 5%) by the International Peripheral T-cell Lymphoma Project. Nasal NK/T-cell lymphoma most commonly occurred in the nasal cavity or other parts of the upper aerodigestive tract, and is highly associated with EBV (Epstein-Barr virus) infection, which is highly aggressive and its prognosis generally is poor. The mean survival time is about 12-38 months. It is important to accurately assess the patients prognosis for an optimal treatment. Localized disease (stage I and II) often has a relatively good prognosis through local radiation or combined therapy. There is a paucity of data to guide therapy in advanced disease. Generally, combining therapy is the most commonly selected approach for advanced disease. The conditions of the patients with advanced, relapsed or refractory diseases have been improved by high-intensity chemotherapy combined with radiation, and a regimen containing L-asparaginase L-Asp. Recently some studies have demonstrated promising outcomes in the selected cases by high-dose chemotherapy supplemented with auto-or allo-HSCT. Targeting therapy is also developing quickly. This current review mainly focuses on the advance of treatment for extranodal NK/T-cell lymphoma.
Antineoplastic Combined Chemotherapy Protocols
;
Hematopoietic Stem Cell Transplantation
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Humans
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Lymphoma, Extranodal NK-T-Cell
;
drug therapy
;
radiotherapy
;
therapy
2.Pretreatment evaluation of 18F-FDG PET-CT in extranodal NK/T-cell lymphoma.
Pan PENG ; Ning WU ; Xiu Li TAO ; Ying LIU ; Lyu LYU ; Xin CHENG
Chinese Journal of Oncology 2022;44(4):370-376
Objective: To investigate the clinical value of pretreatment 18F-fluorodeoxy glucose positron emission tomography/computed tomography (18F-FDG PET-CT) in extranodal NK/T-cell lymphoma. Methods: Eighty-one patients with pathologically confirmed extranodal NK/T-cell lymphoma and pretreatment with PET-CT scan in Cancer Hospital, Chinese Academy of Medical Sciences from August 2006 to December 2017 were enrolled in the study. The clinical, follow-up and imaging data were analyzed retrospectively. The relationship between maximum standard uptake value (SUVmax) and prognosis were evaluated by Mann-Whitney U test and Spearman rank correlation analysis. Results: Among the 81 patients, 98.8% (80/81) were upper aerodigestive tract (UAT) involved. Lesions at extra-UAT sites were detected in 7 cases, involving parotid gland (n=1), breast (n=1), spleen (n=1), pancreas (n=1), skin and subcutaneous soft tissue (n=1), muscle (n=1), lung (n=2) and bone (n=3). Lymph node involvement were demonstrated in 33 cases. All of the lesions had increased uptake of PET, the median SUVmax was 8.6. PET-CT changed staging in 15 cases, and 12 cases were adjusted treatment methods. 21 cases were changed radiotherapy target because of PET-CT. The 1-, 2-year progression-free survival (PFS) rates were 88.7% and 80.3% while 1-, 2-year overall survival (OS) rates were 97.2% and 94.4% respectively. The median SUVmax of patients with local lymph nodes involvement was significantly higher than those without local lymph nodes involvement (P=0.007). The SUVmax was positively associated with Ann Arbor stage (r=0.366, P=0.001), lactate dehydrogenase (r=0.308, P=0.005) and Ki-67 level (r=0.270, P=0.017). The SUVmax was inversely associated with lymphocyte count (r=-0.324, P=0.003) and hemoglobin content (r=-0.225, P=0.043). Conclusions: Extranodal NK/T-cell lymphoma predominantly occurs in extra-nodal organs, mainly in the upper respiratory and gastrointestinal tracts, with marked FDG-addiction. Compared with conventional imaging, 18F-FDG PET-CT is sensitive and comprehensive in detecting extra-nodal NK/T-cell lymphoma involvement, assisting in accurate clinical staging and treatment planning. Pretreatment SUVmax is potential for prognosis evaluation since it is correlated with prognostic factors.
Fluorodeoxyglucose F18
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Humans
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Lymphoma, Extranodal NK-T-Cell/radiotherapy*
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Positron Emission Tomography Computed Tomography/methods*
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Prognosis
;
Radiopharmaceuticals
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Retrospective Studies
3.Curative effect of nasal type extranodal NK/T-cell lymphoma by sequential chemotherapy combined radiotherapy compared with chemotherapy.
Cunbang WANG ; Hai BAI ; Rui XI ; Yaozhu PAN ; Shufen XU ; Qian ZHANG ; Yan CHEN ; Jinmao ZHOU
Journal of Clinical Otorhinolaryngology Head and Neck Surgery 2013;27(23):1283-1290
OBJECTIVE:
To explore the curative effect of nasal type extranodal NK/T-cell lymphoma by sequential chemotherapy combined radiotherapy compared with chemtherapy.
METHOD:
Fifty-seven cases of nasal type extranodal NK/T-cell lymphoma confirmed by pathological morphology and immuno-histochemistry were divided into chemotherapy combined radiotherapy group (34 cases) or chemotherapy group (23 cases). Twenty-three patients in the chemotherapy group alternately applied CHOP, VDLP and MEOP regimen after each two treatments into the clinical observation; Chemotherapy combined radiotherapy group of 34 patients, in addition to the above chemotherapy, applied three-dimensional conformal radiation therapy of the primary site by linear accelerators. Then all of patients were ceased treatment and followed up 3-5 years.
RESULT:
(1) After treatment, effective rate of two groups was 91.2% and 87.0%, there was no obvious difference (P > 0.05); After follow up about 1 year, effective rate of two groups was 76.5% and 73.9%, there was no obvious difference (P > 0.05); (3) After follow up about 3 years and 5 years, disease free survival (DFS) of two groups was 61.3%, 47.6% and 43.5%, 21.4%, there was obvious difference (P < 0.05). (4) Long-term survival is closely related to treatment mode. (5) B symptoms, advanced (III, IV) stage, the International Prognostic Index (IPI), KPS scores were correlated with prognosis, and were independent prognostic factors.
CONCLUSION
Treatment with chemotherapy and radiotherapy for nasal type extranodal NK/T-cell lymphoma had obvious curative effect and may improve long-term survival efficiently compared with chemotherapy alone.
Adult
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Antineoplastic Combined Chemotherapy Protocols
;
Combined Modality Therapy
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Female
;
Humans
;
Lymphoma, Extranodal NK-T-Cell
;
drug therapy
;
radiotherapy
;
therapy
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Male
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Middle Aged
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Radiotherapy
;
methods
;
Treatment Outcome
;
Young Adult
4.Sinonasal natural killer/T-cell lymphoma presenting as pyrexia of unknown origin with nasal symptoms.
Betsy K H SOON ; Xin-Rong LIM ; Deborah H L NG ; Ming-Yann LIM
Singapore medical journal 2014;55(7):e109-11
A 68-year-old Chinese man presented with an eight-month history of pyrexia of unknown origin and chronic sinusitis despite multiple courses of antibiotics. He underwent extensive investigations, including workups for infections, chronic granulomatous diseases and malignancy. Nasal biopsies were performed twice under local anaesthesia, but did not show any evidence of malignancy. Eventually, the patient was diagnosed with natural killer (NK)/T-cell lymphoma, nasal variant, based on histopathological findings from harvested deep tissue obtained via functional endoscopic sinus surgery. This study highlights that, for patients presenting with pyrexia of unknown origin and nasal symptoms, NK/T-cell lymphoma must be considered as a differential diagnosis. Generous amounts of tissue should be harvested under general anaesthesia rather than limited tissue under local anaesthesia, in order to facilitate and ensure a definitive diagnosis.
Aged
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Anti-Bacterial Agents
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chemistry
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Biopsy
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China
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Humans
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Lymphoma, Extranodal NK-T-Cell
;
diagnosis
;
diagnostic imaging
;
pathology
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Male
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Nasal Cavity
;
pathology
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Nose Neoplasms
;
complications
;
diagnosis
;
radiotherapy
;
Prognosis
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Radiography
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Radiotherapy
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Sinusitis
;
complications
;
diagnosis
;
Treatment Outcome
5.The Roles of Radiotherapy and Chemotherapy in the Era of Multimodal Treatment for Early-Stage Nasal-Type Extranodal Natural Killer/T-Cell Lymphoma.
Tae Hyung KIM ; Jin Seok KIM ; Yang Gun SUH ; Jaeho CHO ; Woo Ick YANG ; Chang Ok SUH
Yonsei Medical Journal 2016;57(4):846-854
PURPOSE: To evaluate radiotherapy (RT) and chemotherapy (CT) treatments of early-stage extranodal natural killer/T-cell lymphoma (ENKTL). MATERIALS AND METHODS: Fifty-five patients with stage I or II ENKTL [n=39 (71%) and 16 (29%) patients, respectively] who were treated with RT between 1999 and 2013 were analyzed retrospectively. The median age was 54 years (range, 24-81). Patients were grouped by treatment modality as RT alone [n=19 (35%)], upfront CT plus RT [CT+RT, n=16 (29%)], and concurrent chemoradiotherapy [CCRT, n=20 (36%)]. The median RT dose was 48 Gy. Patient characteristics between each treatment group were well balanced. Patterns of failure and survival were analyzed. RESULTS: The overall response rate after RT was 94.6%. Ten patients experienced distant failure, and seven experienced local failure comprising five in-field and two out-field failures. The local and distant failure rates in the RT-alone group were the same (16%). In the CT+RT group, the most common failure sites were local (19%). In the CCRT group, the most common failures were distant (25%). At a median follow-up of 56 months (range, 1-178 months), the 5-year overall survival (OS) and progression-free survival rates were 66% and 54%, respectively. The 5-year OS rate for the RT-alone and CT+RT groups were 76% and 69%, respectively, and the 2-year OS rate for the CCRT group was 62% (p=0.388). CONCLUSION: In the era of multimodal treatment for ENKTL, RT alone using advanced techniques should be considered for local disease control, whereas maintenance CT regimens should be considered for distant disease control.
Adult
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Aged
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Aged, 80 and over
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Antineoplastic Combined Chemotherapy Protocols/therapeutic use
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*Chemoradiotherapy
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Combined Modality Therapy
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Disease-Free Survival
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Female
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Humans
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Lymphoma, Extranodal NK-T-Cell/*drug therapy/mortality/*radiotherapy
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Male
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Middle Aged
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Retrospective Studies
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Survival Rate
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Treatment Outcome
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Young Adult
6.Expression of natural killer cell development-associated transcription factors in nasal NK/T-cell lymphomas occurring in Chinese population.
Shuang ZHANG ; Lin NONG ; Ying ZHANG ; Ying WANG ; Ting LI
Chinese Journal of Pathology 2010;39(6):366-371
OBJECTIVETo evaluate the expression and prognostic significance of T-bet and its cofactors EOMES, ETS-1 and MEF [which are transcription factors and responsible for development of natural killer (NK) cells] in the extranodal NK/T-cell lymphoma, nasal type (EN-NK/T-NT).
METHODSThe expression status of T-bet, EOMES, ETS-1 and MEF in 40 cases of EN-NK/T-NT occurring in Chinese population was studied by immunohistochemistry and in-situ hybridization (ISH). The clinical relevance was also evaluated. The control cases included 18 cases of peripheral T-cell lymphoma, 10 cases of B-cell lymphoma, 5 cases of normal spleen, 5 cases of normal thymus and 10 cases of nasal mucosal tissues affected by chronic inflammation.
RESULTSThe expression levels of T-bet mRNA and protein were high in EN-NK/T-NT (82.5% and 100%, respectively) and in peripheral T cell lymphoma (17/18 and 72.2%, respectively). There was no expression in B-cell lymphoma. The expression of EOMES (80.0% by ISH), ETS-1 (82.5% by ISH) and MEF (62.5% by ISH) was high in EN-NK/T-NT, but not in the control group. The frequency of co-expression of T-bet and EOMES (75%, 30/40) was significantly higher than that of the other genes. Follow-up study showed that the mean and median survival of the 19 cases of EN-NK/ T-NT was 33 months and 10 months, respectively. The five-year survival rate was 10.5%. Statistical analysis showed that only treatment modalities significantly affected the patients' overall survival; and none of the four transcription factors had significant impact on survival. The expression rates of T-bet, EOMES, ETS-1 or MEF had no significant difference between the 9 alive and the 10 dead cases.
CONCLUSIONSThe expression of T-bet correlates with the lymphoma types. It is mainly expressed in peripheral NK and T-cell lymphomas. The important functional gene engaged in NK cells development is highly expressed in EN-NK/T-NT. They may play a crucial role in pathogenesis and aggressive biologic behavior.
Adolescent ; Adult ; Aged ; China ; epidemiology ; DNA-Binding Proteins ; metabolism ; Female ; Follow-Up Studies ; Humans ; Lymphoma, Extranodal NK-T-Cell ; Lymphoma, T-Cell, Peripheral ; metabolism ; Male ; Middle Aged ; Nose Neoplasms ; drug therapy ; metabolism ; pathology ; radiotherapy ; Proto-Oncogene Protein c-ets-1 ; metabolism ; RNA, Messenger ; metabolism ; Survival Rate ; T-Box Domain Proteins ; genetics ; metabolism ; Transcription Factors ; metabolism ; Young Adult
7.Clinicopathological observation on 2 cases of nasal-type NK/T-cell lymphoma in the esophagus.
Shu ZHANG ; Hai WANG ; Dong-ni LENG
Chinese Journal of Oncology 2008;30(10):767-769
Antineoplastic Combined Chemotherapy Protocols
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therapeutic use
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CD56 Antigen
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metabolism
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Cyclophosphamide
;
therapeutic use
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Doxorubicin
;
therapeutic use
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Esophageal Neoplasms
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drug therapy
;
metabolism
;
pathology
;
radiotherapy
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Follow-Up Studies
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Humans
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Leukocyte Common Antigens
;
metabolism
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Lymphoma, Extranodal NK-T-Cell
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drug therapy
;
metabolism
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pathology
;
radiotherapy
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Male
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Middle Aged
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Poly(A)-Binding Proteins
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metabolism
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Prednisone
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therapeutic use
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T-Cell Intracellular Antigen-1
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Treatment Outcome
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Vincristine
;
therapeutic use
8.Treatment outcome and prognosis of autologous hematopoietic stem cell transplantation combined with high dose radiotherapy/chemotherapy in 22 patients with nasal NK/T cell lymphoma.
Xiu-Zhen CUI ; Hua-Qing WANG ; Xian-Ming LIU ; Hui-Lai ZHANG ; Wei LI
Chinese Journal of Hematology 2007;28(9):609-611
OBJECTIVETo analyze the outcome and prognosis of autologous hematopoietic stem cell transplantation (AHSCT) combined with high dose radiotherapy/chemotherapy in 22 patients with nasal NK/T cell lymphoma.
METHODSFrom July 1992 to December 2005, 22 patients with nasal NK/T cell lymphoma were diagnosed pathologically. Immunophenotyping was performed in 13 cases. The patients were classified by Ann Arbor staging system and international prognosis index (IPI). The patients received cycles of chemotherapy every other two weeks or combined with radiotherapy for remission induction, followed high dose radiotherapy/chemotherapy, combined with autologous peripheral blood stem cell transplantation (APBSCT), or autologous bone-marrow transplantation (ABMT). Patients were given complementary radiotherapy after transplantation if they did not have it before. Twelve patients of IPI 3 -4 received consolidation chemotherapy, and one of them received the second transplantation.
RESULTSThe median follow-up duration was 64 (12 - 168) months. The 5 and 8-year overall survivals (OS) were 79.3% and 64.1%, and disease free survivals (DFS) were 36.4% and 27.3%, respectively. The 5-year OS were as follows: for stage I - II and III - IV disease were 90.0% and 70.0% (P = 0. 041); for patients without and with B symptom were 100.0% and 70.7% (P = 0.045); and for IPI 1 - 2 and 3 - 4 were 100.0% and 60.0% (P = 0.035), respectively. Multivariate analysis by COX regression revealed that disease stage, B symptom and IPI were independent prognostic factors.
CONCLUSIONAHSCT combined with high dose radiotherapy/chemotherapy is an effective treatment for patients with poor prognosis nasal NK/T cell lymphoma.
Adult ; Aged ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Combined Modality Therapy ; Female ; Follow-Up Studies ; Hematopoietic Stem Cell Transplantation ; Humans ; Lymphoma, Extranodal NK-T-Cell ; diagnosis ; radiotherapy ; therapy ; Male ; Middle Aged ; Nose Neoplasms ; diagnosis ; radiotherapy ; therapy ; Prognosis ; Transplantation, Autologous ; Treatment Outcome
9.Prognostic factors and treatment outcome in early stage nasal NK/T cell lymphoma.
Bo YAO ; Ye-xiong LI ; Hui FANG ; Zi-hao YU ; Jing JIN ; Xin-fan LIU
Chinese Journal of Hematology 2006;27(4):222-225
OBJECTIVETo analyze initial response rate of radiotherapy and chemotherapy for early nasal NK/T-cell lymphoma, and its prognostic factors.
METHODSFrom January 1996 to December 2002, 116 patients with nasal NK/T-cell lymphoma were diagnosed pathologically. Immunophenotyping was performed in 50 cases. According to Ann Arbor staging classification, 95 patients were stage I(E) and 21 II(E). Of the 116 patients, 22 received radiotherapy alone, 6 chemotherapy alone and 88 combined modality therapy (CMT), including, 41 radiotherapy followed by chemotherapy, and 47 chemotherapy followed by radiotherapy.
RESULTSThe 5-year overall survival (OS) rate and disease free survival (DFS) rate for all patients was 74.1% and 61.5%, respectively. For stage I(E) and II(E) patients, the 5-year OS rate was 75.1% and 68% (P = 0.45), and DFS rate was 64.7% and 47.8%, respectively (P = 0.07). The 5 year OS rate and DFS rate were 86.5% and 71.5% for patients who achieved complete response (CR), and 18.4% and 17.2% for those who didn't, respectively (P = 0.000). Sixty-three patients were treated with radiotherapy alone or radiotherapy followed by chemotherapy, while 53 with chemotherapy followed by radiotherapy or chemotherapy alone. The CR rate for radiotherapy was 74.6% while for chemotherapy was 20.8% (P = 0.000). The 5-year OS rate and DFS rate were 76.8% and 65.4% for radiotherapy with or without chemotherapy, and 78.8% and 61.8% for chemotherapy followed by radiotherapy (P > 0.05). Multivariate analysis by COX regression showed that CR rate was the only independent prognostic factor.
CONCLUSIONThe CR rate of radiotherapy is much higher than that of conventional chemotherapy. Addition of chemotherapy to radiotherapy do not improve the survival of patients with early stage nasal NK/T-cell lymphoma. Radiotherapy is the primary treatment for stage I and II nasal NK/T-cell lymphoma.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Child ; Combined Modality Therapy ; Drug Therapy ; methods ; statistics & numerical data ; Female ; Follow-Up Studies ; Humans ; Kaplan-Meier Estimate ; Lymphoma, Extranodal NK-T-Cell ; therapy ; Male ; Middle Aged ; Nasal Cavity ; Nose Neoplasms ; therapy ; Prognosis ; Proportional Hazards Models ; Radiotherapy ; methods ; statistics & numerical data ; Treatment Outcome ; Young Adult
10.Mediastinal Single Nodal Relapse of a Nasal Nk/T cell Lymphoma.
Kyoung Hoon RHEE ; Seok Chan HONG ; Jeong Min AN ; Jooryung HUH ; Jin Sook RYU ; Jin Seong LEE ; Cheolwon SUH
The Korean Journal of Internal Medicine 2007;22(3):201-205
A nasal NK/T cell lymphoma is a very aggressive form of lymphoma. Patterns of relapse after treatment have not been systematically evaluated, and mediastinal nodal relapse at a primary site has never been documented. We describe here a 40-year old man who presented with a nasal obstruction caused by a protruding mass that was identified as a nasal NK/T cell lymphoma. The initial work-up, including chest and abdominopelvic computed tomography (CT) and positron emission tomography (PET), showed no regional or distant metastasis. A CT scan performed following three cycles of chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP) showed that the mass had nearly disappeared. Radiation therapy undertaken following chemotherapy was given to the primary site. However, PET performed following radiotherapy revealed a single mediastinal lymphadenopathy, with no evidence of residual tumor in the nasal cavity. A biopsy using video-assisted thoracoscopy (VATS) showed the presence of a recurrent NK/T cell lymphoma with an immunophenotype identical to that of the primary nasal lymphoma. An additional three cycles of CHOP chemotherapy were administered, and the patient remains alive, with no evidence of disease 30 months after the initial relapse. These findings indicate that early detection with PET and prompt surgical excision with the use of VATS can lead to successful treatment of a relapsed nasal NK/T cell lymphoma.
Adult
;
Antineoplastic Combined Chemotherapy Protocols/*administration & dosage
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Combined Modality Therapy
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Cyclophosphamide/administration & dosage
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Doxorubicin/administration & dosage
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Humans
;
Lymphatic Metastasis
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Lymphoma, Extranodal NK-T-Cell/*diagnosis/*therapy
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Male
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Nose Neoplasms/*diagnosis/*therapy
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Positron-Emission Tomography
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Prednisolone/administration & dosage
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Radiotherapy, Adjuvant
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Tomography, X-Ray Computed
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Vincristine/administration & dosage