Early locoregional high-dose radiotherapy is associated with long-term disease control in localized primary angiocentric lymphoma of the nose and nasopharynx

Abstract

Nasal NK/T cell is a rare form of usually localized non-Hodgkin's lymphoma (NHL) which generally carries a poor prognosis when treated with conventional NHL chemotherapy protocols. We reviewed 20 consecutive localized stage I/II nasal NK/T cell lymphomas treated at our institution over a 29 year period. Median age was 44 (range 23-71). Front-line therapy was generally radiotherapy alone (35-70 Gy) before 1980 and combination chemotherapy after 1980. Six patients were treated with first-line radiotherapy and they achieved complete remission (CR). Two subsequently received combination chemotherapy. Five of those patients remained in complete remission, after 97؉ to 277؉ months. Twelve patients were treated with first-line chemotherapy including CHOP or CHOPlike regimen in seven cases, and COP in five cases. Only three of them achieved CR, five had partial response and four had progressive disease. Five of the seven patients treated with CHOP did not achieve complete remission. The nine patients who failed to achieve CR with chemotherapy subsequently received salvage radiotherapy but only two of them obtained CR. Finally, two patients were treated with alternated chemotherapy and radiotherapy and achieved CR, which persisted after 14+ and 26+ months. Median survival was not reached in patients who received front-line radiotherapy, and was 35 months in patients who received front-line chemotherapy. These findings confirm that chemotherapy gives a low complete remission rate in localized nasal NK/T cell lymphoma. By contrast, first-line radiotherapy seems to give favorable results, whereas its results are poorer when administered after resistance to chemotherapy. Whether the use of chemotherapy after radiotherapy, or alternated chemotherapy-radiotherapy regimens give better clinical results than radiotherapy alone will have to be evaluated prospectively in this type of NHL. Leukemia (2001Leukemia ( ) 15, 1123Leukemia ( -1126

Natural killer-cell malignancies: diagnosis and treatment

“…The overall response rate ranged from 60 to 80%, complete remission (CR) rate 40 to 80% and 5-year overall survival (OS) 40 to 59% (Table 2). 14,[33][34][35][36]38,39,46,47 Typically, a total dose of 30-60 Gy is administered. Computerized tomographic or magnetic resonance imaging scans should be used to determine the radiation target volumes.…”

“…46 In 23 patients with various NK-cell lymphomas, multivariate analysis including the presentation EBV DNA, stage, age and LDH level showed that presentation EBV DNA was the only significant factor, with a load of 46.1 Â 10 7 copies/ml negatively impacting on disease-free survival (DFS).…”

Primary nasal natural killer cell lymphoma: long-term treatment outcome and relationship with the International Prognostic Index

“…22 Some patients have been reported from other countries, although their ethnicity is usually poorly defined. [23][24][25][26] Pathologically, NK cell malignancies show a broad morphologic spectrum. The lymphoma infiltrate is often angiocentric, with prominent necrosis and vascular destruction.…”

“…In the revised European-American classification of lymphoid malignancies, these tumors were classified as angiocentric T-cell lymphomas. 29 Although many of the lymphomas reported as polymorphic reticulosis or angiocentric T-cell lymphomas were probably NK cell lymphomas, 10,12,17,19,23,25,26 the lack of information on CD56 and EBV made it difficult to determine if that was in fact the case. Moreover, many clinical studies of nasal lymphomas actually included in their analysis other conventional B-and T-cell lymphomas, which were distinct from NK cell lymphomas.…”

Physiological and Pathological Aspects of Human NK Cells

“…Some patients with early-stage disease are cured by radiation therapy. It has been demonstrated that radiotherapy, either as initial treatment or as part of the chemotherapy regimen, is the single most important key to a successful outcome (Ribrag et al, 2001). However, some patients with early-stage disease have early local or systemic recurrences and die of disease.…”