Zhang Xiao-Ying, Fu Jia, Chen Mei-Ling, Chen Xin-Chun, Zhang Shi-Min, Luo Yi-Ling, Fang Mao, Jiang Han-Wen, Chen Fang, Wang Hao, He Jin-Hua, Li Yan
Department of Pathology, The Affiliated Panyu Central Hospital of Guangzhou Medical University, Guangzhou, 511400, Guangdong, China.
Guangzhou University of Chinese Medicine, Guangzhou, 510006, China.
Endocr Pathol. 2024 Dec;35(4):362-371. doi: 10.1007/s12022-024-09837-w. Epub 2024 Nov 30.
Epstein-Barr Virus (EBV)-positive neuroendocrine carcinoma (NEC) is a rare neoplasm with limited histopathological and therapeutic data. This report presents 22 cases of EBV-positive NEC, analyzing age distribution, morphology, and immunophenotype. The median patient age was 47 years (range: 27-67 years), with a male-to-female ratio of 17:5. Most cases (86%, 19/22) were localized to the nasal cavity or nasopharynx, while the remaining three (14%, 3/22) involved the lung, eyelid, and chest wall. Tumors were identified as small cell neuroendocrine carcinoma (SCNEC) or large cell neuroendocrine carcinoma (LCNEC) based on cellular morphology. Immunohistochemical analysis showed positivity for at least one, but generally, two neuroendocrine markers and INI1, while negativity for NUT and squamous cell carcinoma markers, such as p63, p40, and CK5/6. In situ hybridization consistently revealed EBV early RNAs (EBERs) in all cases. Notably, eight patients benefited from chemoradiotherapy. Recognizing this rare tumor is essential for optimizing treatment strategies.
爱泼斯坦-巴尔病毒（EBV）阳性神经内分泌癌（NEC）是一种罕见肿瘤，其组织病理学和治疗数据有限。本报告介绍了22例EBV阳性NEC病例，分析了年龄分布、形态学和免疫表型。患者中位年龄为47岁（范围：27 - 67岁），男女比例为17:5。大多数病例（86%，19/22）局限于鼻腔或鼻咽部，其余3例（14%，3/22）累及肺、眼睑和胸壁。根据细胞形态，肿瘤被鉴定为小细胞神经内分泌癌（SCNEC）或大细胞神经内分泌癌（LCNEC）。免疫组织化学分析显示至少一种神经内分泌标志物呈阳性，但通常为两种神经内分泌标志物和INI1呈阳性，而NUT以及鳞状细胞癌标志物（如p63、p40和CK5/6）呈阴性。原位杂交在所有病例中均一致显示EBV早期RNA（EBERs）。值得注意的是，8例患者从放化疗中获益。认识到这种罕见肿瘤对于优化治疗策略至关重要。
