Objective: To investigate the clinical, pathological and immunophenotypic features, molecular biology and prognosis of fibrin-associated large B-cell lymphoma (LBCL-FA) in various sites. Methods: Six cases of LBCL-FA diagnosed from April 2016 to November 2021 at the Beijing Friendship Hospital, Capital Medical University, Beijing, China and the First Affiliated Hospital, Wenzhou Medical University, Wenzhou, China were collected. The cases were divided into atrial myxoma and cyst-related groups. Clinical characteristics, pathological morphology, immunophenotype, Epstein Barr virus infection status, B-cell gene rearrangement and fluorescence in situ hybridization of MYC, bcl-2, bcl-6 were summarized. Results: The patients' mean age was 60 years. All of them were male. Three cases occurred in atrial myxoma background, while the others were in cyst-related background, including adrenal gland, abdominal cavity and subdura. All cases showed tumor cells located in pink fibrin clot. However, three cyst-related cases showed the cyst wall with obviously fibrosis and inflammatory cells. All cases tested were non germinal center B cell origin, positive for PD-L1, EBER and EBNA2, and were negative for MYC, bcl-2 and bcl-6 rearrangements, except one case with MYC, bcl-2 and bcl-6 amplification. All of the 5 cases showed monoclonal rearrangement of the Ig gene using PCR based analysis. The patients had detailed follow-ups of 9-120 months, were treated surgically without radiotherapy or chemotherapy, and had long-term disease-free survivals. Conclusions: LBCL-FA is a group of rare diseases occurring in various sites, with predilection in the context of atrial myxoma and cyst-related lesions. Cyst-related lesions with obvious chronic inflammatory background show more scarcity of lymphoid cells and obvious degeneration, which are easy to be missed or misdiagnosed. LBCL-FA overall has a good prognosis with the potential for cure by surgery alone and postoperative chemotherapy may not be necessary.
目的： 探讨发生于不同部位的纤维素相关大B细胞淋巴瘤（LBCL-FA）临床、病理形态、免疫表型、分子生物学和预后。 方法： 收集首都医科大学附属北京友谊医院和温州医科大学附属第一医院2016年4月至2021年11月诊断的6例LBCL-FA病例，分为心房黏液瘤和囊肿相关2组病例，总结临床特征、病理形态、免疫表型、EB病毒感染、基因重排和MYC、bcl-2、bcl-6的荧光原位杂交检测结果。 结果： 6例LBCL-FA，均为男性患者，平均年龄为60岁；发生于心房黏液瘤背景3例和囊肿相关背景3例（肾上腺、腹腔和硬膜下）；6例均可见肿瘤细胞分布于粉染纤维素物质中，其中囊肿相关病例的囊壁明显纤维化伴炎性细胞浸润；肿瘤细胞起源于生发中心外活化B细胞，一致性表达PD-L1、EB病毒编码的RNA（EBER）和EB病毒核抗原2（EBNA2）。心房黏液瘤的病例中CD30阳性率高于囊肿相关病例。肿瘤细胞均未检测到MYC、bcl-2和bcl-6基因断裂，仅1例伴有MYC、bcl-2和bcl-6基因异常扩增信号；5/5例免疫球蛋白（Ig）基因聚合酶链反应（PCR）检测到克隆性重排。所有患者随访期9~120个月，手术治疗后未行放化疗长期无病生存。 结论： LBCL-FA是一组发生于不同部位的罕见疾病，好发生于心房黏液瘤和囊肿相关病变的背景中。囊肿相关病变具有显著的慢性炎性背景，肿瘤细胞更为稀疏且退变明显，容易漏诊、误诊。LBCL-FA临床预后好，可以手术完全切除的病例，不一定要进行放化疗。.