Department of Radiation Oncology, The University of Texas M.D. Anderson Cancer Center, Houston, TX.
Department of Radiation Oncology, The University of Texas M.D. Anderson Cancer Center, Houston, TX.
Clin Lymphoma Myeloma Leuk. 2019 Jun;19(6):381-389. doi: 10.1016/j.clml.2019.02.011. Epub 2019 Feb 26.
Extranodal follicular lymphoma (E-FL) is a rare entity that has distinct characteristics and outcomes compared with nodal follicular lymphoma.
This cohort comprised 37 patients with stages I/II E-FL, diagnosed from 2003 to 2013. Outcomes included progression-free survival (PFS), and overall survival (OS). Survival outcomes were calculated using Kaplan-Meier methods.
Median age was 60 years (range, 37-84 years). Disease was stage I in 29 (78.4%). The Follicular Lymphoma International Prognostic Index score was 0 to 1 in 31 (83.8%), 2 in 2 (5.4%), 3 in 1 (2.7%), and missing in 3 (8.1%). Sites of involvement included the gastrointestinal (GI) tract in 22 (59.5%), and non-GI sites in 15 (40.5%). Initial management consisted of chemotherapy (CHT) alone in 21 (56.8%), radiation therapy (RT) alone in 2 patients (5.4%), RT and rituximab in 1 (2.7%), CHT and RT in 7 (18.9%), and observation in 6 (16.2%). RT was to a median dose of 30.6 Gy (range, 23.4-44.0 Gy). At a median follow-up of 69 months (range, 8-157 months), 5-year PFS and OS were 70.4% and 94.4%, respectively. Although the 5-year PFS of those observed was worse than for those who received therapy (33.3% vs. 77.6%; P = .011), that did not translate into an OS difference. Patients who received RT as part of upfront management had a 100% local control (LC) rate and a trend toward improved 5-year PFS (90% vs. 62.2%; P = .067).
Early stage E-FL is an indolent disease and is associated with excellent OS. Treatment strategies should be individualized with RT prioritized when LC is a significant goal.
结外滤泡性淋巴瘤（E-FL）是一种罕见的实体瘤，与结内滤泡性淋巴瘤相比，具有独特的特征和预后。
本队列包括 37 例 2003 年至 2013 年间诊断为 I/II 期 E-FL 的患者。结局包括无进展生存期（PFS）和总生存期（OS）。使用 Kaplan-Meier 方法计算生存结局。
中位年龄为 60 岁（范围，37-84 岁）。29 例（78.4%）为 I 期疾病。滤泡性淋巴瘤国际预后指数评分 0-1 分 31 例（83.8%），2 分 2 例（5.4%），3 分 1 例（2.7%），3 分缺失 3 例（8.1%）。受累部位包括胃肠道（GI）22 例（59.5%）和非-GI 部位 15 例（40.5%）。初始治疗包括单纯化疗（CHT）21 例（56.8%），单纯放疗（RT）2 例（5.4%），RT 和利妥昔单抗 1 例（2.7%），CHT 和 RT 7 例（18.9%），观察 6 例（16.2%）。RT 中位数剂量为 30.6 Gy（范围，23.4-44.0 Gy）。中位随访 69 个月（范围，8-157 个月）时，5 年 PFS 和 OS 分别为 70.4%和 94.4%。尽管观察治疗组 5 年 PFS 差于治疗组（33.3% vs. 77.6%；P=0.011），但并未转化为 OS 差异。作为初始治疗一部分接受 RT 的患者局部控制率为 100%，5 年 PFS 有改善趋势（90% vs. 62.2%；P=0.067）。
早期 E-FL 是一种惰性疾病，与极好的 OS 相关。治疗策略应个体化，当局部控制是一个重要目标时，应优先考虑 RT。
