Purpose: To examine, retrospectively, national-wide clinical data of patients with localized extranodal non-Hodgkin's lymphoma (NHL) who were treated by radiation therapy with or without chemotherapy. Materials and Methods: The survey was carried out at 25 radiation oncology institutions in Japan in 1998. In 1999, according to the REAL classification, central pathological review conducted at Aichi cancer center was carried out for the data from 7 radiation oncology institutions. The 5-year progression free survival rates (PFS) were calculated to identify prognostic factors.
Results: 1) Survey: Data from 1, 141 patients with stage I and II NHL were recruited from 1988 through 1992. Of them, 787 patients, who were treated using definitive radiotherapy with or without chemotherapy for intermediate and high-grade lymphomas in Working Formulation, constituted the core of this study. Primary tumors arose mainly from extra-nodal organs (71%) in the head and neck (Waldeyer's ring: 41%, thyroid gland: 7%, nasal cavities: 5%, oral cavities: 4%, sinus: 3%, orbital structures: 3%, skin: 2% and etc.). The median age of 60 years for patients with extra-nodal NHL was higher than that of 56 years for patients with nodal NHL (p<0.01). Female were dominant in incidence of extra-nodal NHL arising from the thyroid gland, skin and gastrointestinal tract. The percentage of stage I to the extra-nodal NHL from orbit, sino-nasal presentation was higher than that of other NHLs. The percentage of stage II to the extra-nodal NHL from Waldeyer's ring and thyroid gland was higher than that of other NHLs.
2) Central pathological review was carried out for pathological data from 79 patients (Waldeyer's ring: 45, thyroid gland: 19, sinonasal cavities: 15). Of these, diffuse large B cell lymphoma (DLBCL) composed 63% of all patients, mucosa associated lymphoid tissue lymphoma (MALT-L): 16%, Natural Killer/T cell lymphoma (NK/T-L): 11%, and mantle cell lymphoma: 5% in REAL, respectively. In the case of Waldeyer's ring, DLBCL was dominant (80%) in incidence. In the case of thyroid gland. MALT-L was 52% of the total and in the case of sinonasal cavity NK/'I-L was 60% of the total in incidence. According to the REAL classification, the 5-year PFS rates for patients with DLBCL, MALT-L, NK/T-L and mantle cell lymphoma were 64%, 83%, 50% and 25%, respectively (p<0.06). On the other hand, according to the Working Formulation, the 5-year PFS rates for patients with follicular lymphoma, diffuse small cleaved cell lymphoma, diffuse mixed cell lymphoma, diffuse large cell lymphoma and immunoblastic lymphoma were 71%. 57%, 79%. 56% and 50%, respectively (p<0.73). The 5-year PFS rates for patients with Waldeyer's ring NHL, thyroid NHL and sinonasal NHL were 61%, 49% and 74%, respectively (p=0.34). The 5-year PFS rates for patients treated with radiation therapy alone and combined modality were 36% and 75%, respectively (p<0.01). The 5-year PFS rates for patients with low grade of IPI and low-intermediate grade were 70% and 59%, respectively (p<0.01). There was no difference of PFS according to the dose and fields of radiotherapy.
Conclusion: The central pathological review was very important in conducting the multi-center clinical trials and multi-institutional surveys. The REAL classification and IPI were useful in predicting the survival of patients with localized extra-nodal NHL.
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