Krol A D, Hermans J, Dawson L, Snijder S, Wijermans P W, Kluin-Nelemans H C, Kluin P M, van Krieken J H, Noordijk E M
Department of Radiotherapy, University Hospital Rotterdam/Daniel den Hoed Cancer Center, The Netherlands.
Cancer. 1998 Oct 15;83(8):1612-9. doi: 10.1002/(sici)1097-0142(19981015)83:8<1612::aid-cncr17>3.0.co;2-g.
Primary extranodal lymphomas (EN-NHLs) are a heterogeneous category of tumors that are considered to be different from primary nodal non-Hodgkin's lymphomas (N-NHLs). To what extent these differences have clinical implications is currently not very clear, because knowledge of EN-NHL as a separate group is limited.
Using data from the Comprehensive Cancer Centre West (CCCW) population-based NHL registry in the Netherlands, N-NHL and EN-NHL patients were compared to determine differences in characteristics at diagnosis, responses to treatment, patterns of failure, and survival.
At presentation, EN-NHL patients had poorer performance scores and more often bulky tumors compared with N-NHL patients, resulting in poorer responses to treatment (complete response rates were 72% and 84%, respectively; P=0.04) and inferior 5-year overall survival (49% and 63%, respectively; P=0.003). Among EN-NHL patients, considerable variations in response, survival, and relapse rates were observed, with gastric NHL patients having the best and central nervous system NHL patients having the worst prognosis (66% and 7% 5-year overall survival, respectively). Relapse rates for N-NHL and EN-NHL patients did not differ (39% and 36% 5-year relapse rates, respectively), whereas among EN-NHL patients considerable differences in relapse rates were noted. Relapses among N-NHL patients were mainly found in nodal sites, whereas recurrent disease in EN-NHL patients was mainly found in extranodal sites.
In this population-based study, Stage I EN-NHL patients as a group had a poorer prognosis than N-NHL patients. However, among EN-NHL patients, considerable differences in response, relapse risk, and survival were observed. The failure analysis conducted in this study suggests that patterns of dissemination for N-NHL and EN-NHL are different.
原发性结外淋巴瘤（EN-NHL）是一类异质性肿瘤，被认为与原发性结内非霍奇金淋巴瘤（N-NHL）不同。目前尚不清楚这些差异在多大程度上具有临床意义，因为作为一个独立组别的EN-NHL的相关知识有限。
利用荷兰西部综合癌症中心（CCCW）基于人群的非霍奇金淋巴瘤登记处的数据，对N-NHL和EN-NHL患者进行比较，以确定诊断时的特征、治疗反应、失败模式和生存率的差异。
初诊时，与N-NHL患者相比，EN-NHL患者的体能状态评分较差，肿瘤体积较大的情况更常见，导致治疗反应较差（完全缓解率分别为72%和84%；P=0.04），5年总生存率较低（分别为49%和63%；P=0.003）。在EN-NHL患者中，观察到反应、生存和复发率存在相当大的差异，胃NHL患者预后最好，中枢神经系统NHL患者预后最差（5年总生存率分别为66%和7%）。N-NHL和EN-NHL患者的复发率没有差异（5年复发率分别为39%和36%），而在EN-NHL患者中，复发率存在相当大的差异。N-NHL患者的复发主要发生在结内部位，而EN-NHL患者的复发性疾病主要发生在结外部位。
在这项基于人群的研究中，I期EN-NHL患者作为一个整体的预后比N-NHL患者差。然而，在EN-NHL患者中，观察到反应、复发风险和生存存在相当大的差异。本研究进行的失败分析表明，N-NHL和EN-NHL的播散模式不同。
