Department of Pathology, Sanford Health, Fargo, ND, USA.
Department of Hematology and Oncology, Sanford Health, Fargo, ND, USA.
Ear Nose Throat J. 2022 Jul;101(6):386-391. doi: 10.1177/0145561320955125. Epub 2020 Sep 30.
Sinonasal lymphoepithelial carcinoma (SLEC) is an extremely rare malignancy. We present a case of SLEC in a 77-year-old man who presented with nasal congestion and persistent sinusitis. Imaging revealed a large right nasal mass involving right paranasal sinuses along with bulky bilateral cervical lymphadenopathy. In addition, there was a fluorodeoxyglucose avid L1 vertebral lesion. Biopsy of nasal mass and cervical lymph nodes showed syncytial growth of tumor cells in a lymphoplasmacytic background. Immunohistochemical stains showed positivity for pankeratin, CK5/6, epithelial membrane antigen, p40 (focal), and p63 (focal). An Epstein-Barr virus-encoded RNA by in situ hybridization was strong and diffusely positive. Based on these pathologic findings and considering the location of tumor, diagnosis of SLEC was rendered. L1 vertebral body lesion was clinical and radiologically considered to be a metastasis. Correlation with radiology to determine the exact location of tumor is extremely important for correct diagnosis due to its histopathologic similarities with relatively more common undifferentiated type of nasopharyngeal carcinoma. No standard treatment protocol has been established for this tumor yet. To our knowledge, this is first ever report of SLEC presented with clinical stage IV disease.
鼻窦黏膜相关淋巴组织边缘区 B 细胞淋巴瘤（MALT 型）是一种极为罕见的恶性肿瘤。我们报告了一例 77 岁男性鼻窦黏膜相关淋巴组织边缘区 B 细胞淋巴瘤患者，其表现为鼻塞和持续性鼻窦炎。影像学检查显示右侧鼻腔有一个大肿块，累及右侧鼻旁窦，并伴有双侧大量颈部淋巴结病。此外，L1 椎体还有氟脱氧葡萄糖摄取活跃的病变。鼻肿块和颈部淋巴结活检显示肿瘤细胞在淋巴浆细胞背景下呈合胞体生长。免疫组化染色显示 pankeratin、CK5/6、上皮膜抗原、p40（局灶性）和 p63（局灶性）阳性。原位杂交显示 Epstein-Barr 病毒编码的 RNA 呈强弥漫阳性。基于这些病理发现，并考虑到肿瘤的位置，诊断为鼻窦黏膜相关淋巴组织边缘区 B 细胞淋巴瘤。L1 椎体病变在临床和影像学上被认为是转移灶。由于其组织病理学与更为常见的未分化型鼻咽癌相似，因此与影像学进行相关性分析对于正确诊断极其重要。目前尚未为该肿瘤建立标准的治疗方案。据我们所知，这是首例报告以临床 IV 期疾病表现的鼻窦黏膜相关淋巴组织边缘区 B 细胞淋巴瘤。
