Conconi Annarita, Janikova Andrea, Vannata Barbara, Ramírez-Ibarguen Ana Florencia, Lobetti-Bodoni Chiara, Belada David, Pirosa Maria Cristina, Mian Michael, Ferreri Andrés J M, Ryan Gail, Pangalis Gerassimos, Cabrera Maria Elena, Luminari Stefano, Montoto Silvia, Tsang Richard, Aurer Igor, Visco Carlo, Casaluci Gloria Margiotta, Prochazka Vit, Hricko Samuel, Stathis Anastasios, Mazzucchelli Luca, Ponzoni Maurilio, Federico Massimo, Gaidano Gianluca, Lopez-Guillermo Armando, Pro Barbara, Rossi Davide, Cascione Luciano, Nowakowsky Grzegorz, Trneny Marek, Zucca Emanuele
Azienda sanitaria locale, Ematologia, Ospedale degli Infermi, Biella, Italy.
Department of Hematology and Oncology, University Hospital Brno, Brno, Czech Republic.
Hematol Oncol. 2025 Jul;43(4):e70111. doi: 10.1002/hon.70111.
The characteristics at diagnosis and clinical course of primary extranodal follicular lymphoma (EFL) have not been extensively described. The International Extranodal Lymphoma Study Group (IELSG) conducted an international retrospective survey aimed to describe the clinical features at diagnosis and the outcomes of FL cases with a clinically dominant extranodal component. The dataset included 605 pathologically reviewed cases from 19 different countries, and their outcomes were compared to those of nodal follicular lymphomas. The two most common presentation sites for EFL were the skin (n = 334) and the gastrointestinal tract (n = 72), with 22 cases having primary duodenal localization. These subsets exhibited unique features at diagnosis and significantly different overall survival (OS) patterns. After a median follow-up of 5.5 years, primary cutaneous lymphomas showed a superior outcome [10-year OS: 89% (95% CI, 83%-93%)], while primary gastrointestinal lymphomas had an intermediate outcome [10-year OS: 79% (95% CI, 59%-90%)]. Among the gastrointestinal lymphomas, primary duodenal lymphomas tended toward the best outcome [10-year OS: 95% (95% CI, 69%-99%)]. Other primary extranodal sites had inferior outcomes [10-year OS: 59% (95% CI, 48%-68%)], similar to primary nodal lymphomas [10-year OS: 57% (95% CI, 49%-64%)]. These findings support the identification of specific primary FL localizations as distinct entities with particular clinical and biological characteristics.
原发性结外滤泡性淋巴瘤（EFL）的诊断特征及临床病程尚未得到广泛描述。国际结外淋巴瘤研究组（IELSG）开展了一项国际回顾性调查，旨在描述诊断时的临床特征以及具有临床显性结外成分的滤泡性淋巴瘤（FL）病例的转归。该数据集包括来自19个不同国家的605例经病理检查的病例，并将其转归与结内滤泡性淋巴瘤的转归进行比较。EFL最常见的两个受累部位是皮肤（n = 334）和胃肠道（n = 72），其中22例为原发性十二指肠受累。这些亚组在诊断时表现出独特特征，总生存（OS）模式也有显著差异。中位随访5.5年后，原发性皮肤淋巴瘤显示出较好的转归[10年OS：89%（95%CI，83%-93%）]，而原发性胃肠道淋巴瘤的转归居中[10年OS：79%（95%CI，59%-90%）]。在胃肠道淋巴瘤中，原发性十二指肠淋巴瘤的转归倾向于最佳[10年OS：95%（95%CI，69%-99%）]。其他原发性结外部位的转归较差[10年OS：59%（95%CI，48%-68%）]，与原发性结内淋巴瘤相似[10年OS：57%（95%CI，49%-64%）]。这些发现支持将特定的原发性FL受累部位识别为具有特定临床和生物学特征的不同实体。
