Chen Wang-Xing, Wu Jun, He Jian-Guo
Department of Otolaryngology, The Fourth Affiliated Hospital, Zhejiang University School of Medicine, Yiwu, 322000, Zhejiang, China.
Curr Mol Med. 2025;25(4):506-510. doi: 10.2174/0115665240272361231229122203.
Large B-cell lymphoma (LBCL) with interferon regulatory factor 4 (IRF4) rearrangement (LBCL-IRF4) is a rare subtype of LBCL, with a high prevalence in Waldeyer's ring as well as the neck, head and gastrointestinal lymph nodes.
A patient with 2-month clinical symptoms of nasal obstruction and facial swelling was reported in this short review. A nasal endoscopy examination revealed a neoplasm in the inferior nasal meatus. Both CT and enhanced MRI showed that a soft tissue occupied the nasolacrimal duct, with bone destruction, and extended into the left nasal cavity and left lacrimal gland area. Then, a biopsy of the neoplasm in the inferior nasal meatus was performed.
HE staining results showed that neoplastic cells presented diffuse growth patterns, abundant cytoplasm, vacuole shape, lightly stained nuclei, and irregular nuclear membrane. Immunohistochemistry staining results revealed MUM1(+), Bcl- 6(+), CD20(+), CD79α(+), and CD10(+). FISH analyses detected positive IRF4 rearrangement. LBCL-IRF4 was diagnosed in the patient. The patient received treatment with four cycles of R-CHOP and two times of rituximab, followed up for 2 years, and finally got complete remission.
For the first time, we summarize the imaging and pathological features, drug treatment, and curative effect of LBCL-IRF4 in the nasolacrimal duct.
伴有干扰素调节因子4(IRF4)重排的大B细胞淋巴瘤(LBCL-IRF4)是LBCL的一种罕见亚型,在Waldeyer环以及颈部、头部和胃肠道淋巴结中具有较高的患病率。
在本简短综述中报告了一名有2个月鼻塞和面部肿胀临床症状的患者。鼻内镜检查发现下鼻道有肿瘤。CT和增强MRI均显示软组织占据鼻泪管,伴有骨质破坏,并延伸至左侧鼻腔和左侧泪腺区域。然后,对下鼻道的肿瘤进行了活检。
HE染色结果显示肿瘤细胞呈弥漫性生长模式,胞质丰富,呈空泡状,核淡染,核膜不规则。免疫组化染色结果显示MUM1(+)、Bcl-6(+)、CD20(+)、CD79α(+)和CD10(+)。FISH分析检测到IRF4重排阳性。该患者被诊断为LBCL-IRF4。患者接受了4个周期的R-CHOP和2次利妥昔单抗治疗,随访2年,最终完全缓解。
我们首次总结了鼻泪管LBCL-IRF4的影像学和病理特征、药物治疗及疗效。