Extranodal natural killer/T-cell lymphoma (ENKL), a subtype of natural killer/T-cell malignancies, is a rare subset of lymphomas with significant biological and clinical heterogeneity. The prognosis of ENKL is variable and therapeutic approaches are not well established. The optimal dose, combination, and sequence of radiotherapy and chemotherapy are evolving, as is the role of stem cell transplantation. Radiotherapy is an essential component of therapy for early-stage disease. The clinical course of advanced disease is highly aggressive, with frequent chemotherapy resistance and a poor prognosis. For relapsed disease, asparaginase-based regimens have provided encouraging results and are currently under investigation in the frontline setting. Our article discusses the key aspects of biology, pathogenesis and clinical presentation that contribute to the heterogeneity, and proposes a stratified approach to the treatment of ENKL based on clinical, pathologic and biologic risk factors. Although considerable advances have been made in our understanding of the biology and prognosis of this lymphoma, it remains critical that all patients with a diagnosis of ENKL are enrolled and treated in clinical trials so that optimal therapies can be identified.
"The standard treatment for extranodal NK/T-cell lymphoma, nasal type, is RT combined with chemotherapy. Various factors, including age, B symptoms, performance status, an elevated LDH level, and regional lymph node involvement, are known to affect the survival outcome . Patients with stage I disease and without the risk factors can be treated with RT alone, whereas those with stage I disease and poor risk factors or stage II disease can be treated with SCRT or CCRT . "
[Show abstract][Hide abstract] ABSTRACT: The purpose of this report is to summarize our clinical experience of patients with stage I/II extranodal natural killer (NK)/T-cell lymphoma, nasal type, treated using sequential chemotherapy followed by radiotherapy (SCRT) or concurrent chemoradiotherapy (CCRT).
Forty-three patients with stage I/II extranodal NK/T-cell lymphoma, nasal type, who received SCRT (16 patients) or CCRT (27 patients) were included in the present analysis.
The median follow-up time was 39 months (range, 4-171 months) for all patients, 77 months (range, 4-171 months) for the SCRT group, and 31 months (range, 6-132 months) for the CCRT group. There were no statistically significant differences between the SCRT and CCRT groups with regard to the 3-year progression-free survival (PFS) (56% vs. 41%, P=0.823) and 3-year overall survival (OS) (75% vs. 59%, P=0.670). Univariate analysis revealed that patients with tumors confined to the nasal cavity and patients achieved complete remission had better PFS and OS rates, regardless of the treatment sequence. Multivariate analysis revealed that patients with tumors confined to the nasal cavity and patients aged ≤60 years had better OS rates.
The effect of SCRT and CCRT are similar in terms of survival outcomes of patients with stage I/II extranodal NK/T-cell lymphoma, nasal type. Our results show that tumors confined to the nasal cavity and an age ≤60 years were associated with a better prognosis.
Blood Research 12/2013; 48(4):274-81. DOI:10.5045/br.2013.48.4.274
[Show abstract][Hide abstract] ABSTRACT: Nasal NK/T-cell lymphoma is a rare presentation of T-cell lymphoma in USA and in Europe, but is the most common presentation in Latin America. The lymphoma is associated with a worse prognosis even in the early stage. Until now, a better treatment has not been determined. We performed a prospective, open-label, controlled clinical trial to assess the efficacy and toxicity of the most common treatment options. We treated 427 patients, of whom 109 patients received radiotherapy (RT), 116 patients received chemotherapy (C), and 202 patients received combined therapy (CT), which were balanced according to stage and prognostic factors. Complete response was achieved in 91 % (95 % confidence interval CI 88-102 %) in CT arm 69 % (95 % CI 61-75 %) in RT arm; and 59 % (95 % CI 48-64 %) in C arm (p < 0.01). A progression-free disease was 91 % (95 % CI 83-96 %); 78 % (95 % CI 69-86 %); and 40 % (95 % CI 32-46 %), respectively (p < 0.01). Actuarial curves of overall survival at 5 years were as follows: 86 % (95 % CI 81-90 %), for CT; 64 % (95 % CI 59-70 %) for RT; and 45 % (95 % CI 39-51 %) for C (p < 0.001). Toxicity was mild and well tolerated. To our knowledge, this is the first controlled clinical trial, with a large number of patients and longer follow-up. Thus, we conclude that CT is the best therapeutic option in this setting of patients.
Medical Oncology 09/2013; 30(3):637. DOI:10.1007/s12032-013-0637-1 · 2.63 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Natural killer (NK)/T-cell lymphoma are part of lymphoproliferative diseases, they are rare in Europe and the United States but relatively common in Asia and South America. Natural killer (NK)/T-cell lymphoma present clinically as destructive lesions of the upper aerodigestive tract with perforation of deep structures of the face, destruction of the palate or an invasion of orbits. Treatment modalities of these lymphomas are still discussed because of the lack of available studies and the rarity of this disease. Radiotherapy provides a rapid control of the disease and is positioned as a major treatment of localized stages of NK/T-cell lymphoma of the nasal cavity. We therefore studied the different forms of radiation therapy in the early stages of nasal NK/T-cell lymphoma.
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