Mian M, Capello D, Ventre M B, Grazio D, Svaldi M, Rossi A, Tsang R, Gospodarowicz M K, Oldani E, Federico M, Luminari S, Marcheselli L, Pogliani E M, Rossini F, Cabrera M E, Martelli M, Gutierrez-Garcia G, Busetto M, Visco C, Fiegl M, Rossi D, Gaidano G, Cavalli F, Zucca E, Rambaldi A, Cortelazzo S
Division of Hematology, Azienda Ospedaliera S. Maurizio, Bolzano/Bozen, Italy,
Ann Hematol. 2014 Feb;93(2):221-231. doi: 10.1007/s00277-013-1856-4. Epub 2013 Aug 20.
It is known that extranodal head and neck diffuse large B cell lymphomas (eHN-DLBCL) can affect various anatomical structures what is not well-known, however, is whether they differ in terms of clinical presentation and outcome. Clinical data of the multi-institutional series, the largest of its kind as yet, has been analysed with the aim of answering these open questions and providing long-term follow-up information. Data from 488 patients affected by stage I/II eHN-DLBCL was collected: 300 of the Waldeyer's Ring (WR), 38 of the parotid and salivary glands (PSG), 48 of the thyroid gland (TG), 53 of the nasal cavity and paranasal sinuses (NPS), 24 of the palate and oral cavity (POC) and 25 with more than one involved site. Different eHN-DLBCL arising have distinct characteristics at presentation. The intermediate high risk-modified IPI was 67 % in TG, 44 % in WR, 38 % in PSG and POC and 20 % in MS. The worst 5-year survival rate had TG-DLBCL (61 %) due to the 61 % of patients with a mIPI >1. The addition of radiotherapy (cRT) to remitters did not translate into a survival advantage (5-year disease-free survival of 67 % in the cRT group vs. 70 % in the other). Three of four central nervous system recurrences occurred in NPS-DLBCL. Survival of HN-DLBCL was inferior to nodal DLBCL. This study showed that eHN-DLBCL remitters have an inferior survival when compared to nodal DLBCL, and that the addition of cRT does not provide a survival advantage. Since the standard of care nowadays is chemo-immunotherapy, survival of these patients might have been improved.
已知结外头颈部弥漫性大B细胞淋巴瘤（eHN-DLBCL）可累及各种解剖结构，但它们在临床表现和预后方面是否存在差异尚不清楚。为了回答这些悬而未决的问题并提供长期随访信息，我们分析了同类中规模最大的多机构系列临床数据。收集了488例I/II期eHN-DLBCL患者的数据：300例位于瓦尔代尔环（WR），38例位于腮腺和唾液腺（PSG），48例位于甲状腺（TG），53例位于鼻腔和鼻窦（NPS），24例位于腭和口腔（POC），25例有多个受累部位。不同部位发生的eHN-DLBCL在临床表现上具有不同特征。TG组中高危改良国际预后指数（IPI）为67%，WR组为44%，PSG和POC组为38%，MS组为20%。TG-DLBCL的5年生存率最差（61%），因为61%的患者mIPI>1。对缓解者加用放疗（cRT）并未转化为生存优势（cRT组5年无病生存率为67%，其他组为70%）。四例中枢神经系统复发中有三例发生在NPS-DLBCL。HN-DLBCL的生存率低于结内DLBCL。本研究表明，与结内DLBCL相比，eHN-DLBCL缓解者的生存率较低且加用cRT并未提供生存优势。由于目前的标准治疗是化疗免疫疗法，这些患者的生存率可能已得到改善。
