摘要目的:探讨鼻腔鼻窦肾细胞样腺癌的临床病理特征、免疫表型、鉴别诊断及预后。方法:回顾性分析2014年8月至2018年12月复旦大学附属眼耳鼻喉科医院诊断鼻腔鼻窦上皮性恶性肿瘤（癌）病例，筛选肾细胞样腺癌行临床病理特征分析，并复习相关文献。结果:鼻腔鼻窦上皮性恶性肿瘤（癌）共计460例，其中腺癌70例，占比15.2%。鼻腔鼻窦肾细胞样腺癌仅5例，占比1.1%。其中4例为男性，1例为女性，平均年龄46.5岁（29~52岁），临床表现主要为鼻塞、鼻出血。鼻内镜下为红色息肉样肿块。影像学显示鼻腔、筛窦肿块，伴周围结构的侵犯及骨质破坏。镜下肿瘤细胞呈巢状、腺泡状、微囊结构，间隔以丰富的毛细血管，伴出血，细胞胞质透亮，核级低，核仁不明显，部分区域肿瘤侵及骨组织。免疫组织化学标志物广谱细胞角蛋白（CKpan）、细胞角蛋白（CK）7、碳酸酐酶Ⅸ（CA9）、S-100蛋白、波形蛋白阳性，Ki-67阳性指数低。余肾细胞癌标志物（RCC）、CD10、PAX8、p63、平滑肌肌动蛋白（SMA）、HHF35、Calponin、CD117、甲状腺转录因子（TTF）1及神经内分泌标志物突触素、CHG均阴性。荧光原位杂交（FISH）均未检测到EWSR1和ETV6基因重排。5例患者初诊后均手术切除，其中1例术后二次复发后再次手术切除并行辅助放疗，1例术后行放疗，1例复发后再次手术切除，1例无复发，1例复发后行放疗。随访截至2019年12月，目前5例患者均无瘤生存，一般情况可。结论:原发性鼻腔鼻窦肾细胞样腺癌是低级别非肠型腺癌的一种特殊亚型，发生率低，呈惰性生物学行为，诊断思路属于排他性诊断，需与原发及转移性肿瘤的透明细胞亚型鉴别，免疫组织化学有助于诊断和鉴别诊断，手术切除是主要的治疗方式，可辅助放疗。

abstractsObjective:To investigate the clinicopathological features, immunophenotype, differential diagnosis and prognosis of sinonasal renal cell-like adenocarcinoma.Methods:Retrospective analysis was performed on the cases of sinonasal carcinoma from August 2014 to December 2018 at Eye, Ear, Nose and Throat Hospital, Fudan University. Renal cell-like adenocarcinoma was screened for clinicopathologic feature analysis, and relevant literatures were reviewed.Results:There were 460 cases of sinonasal carcinoma, among which 70 cases (15.2%) were adenocarcinoma, with five (1.1%) being renal cell-like adenocarcinomas. Four patients were male and one was female, with a mean age of 46.5 years (range 29-52 years). The main clinical manifestations were nasal obstruction and epistasis. A red polypoid mass was found under nasal endoscopy. Imaging showed nasal cavity and ethmoid sinus mass with invasion into surrounding structures and bone destruction. Microscopically, the tumor cells were arranged in nests, alveoli and microcapsules with abundant intervening capillaries, accompanied by hemorrhage. The cytoplasm of the cells was clear with low nuclear grade, and the nucleoli were inconspicuous. In some areas, the tumor invaded bone tissue. Immunohistochemical markers CKpan, CK7, CAⅨ, S-100 and vimentin were positive, with low Ki-67 proliferation index. RCC, CD10, PAX8, p63, SMA, HHF35, Calponin, CD117, TTF-1 and neuroendocrine markers Syn and CHG were all negative. EWSR1 and ETV6 gene rearrangements were not detected by FISH. All five patients underwent surgical resection after initial diagnosis. One patient underwent surgical resection after second recurrence and adjuvant radiotherapy, one patient received postoperative radiotherapy, one patient underwent surgical resection after recurrence, one patient had no recurrence and one patient received radiotherapy after recurrence. All five patients had no distant metastasis and survived without tumor up to December 2019.Conclusions:Primary sinonasal renal cell-like adenocarcinoma is a special subtype of low-grade non intestinal adenocarcinoma, with low incidence and inert biologic behavior. At present, most of the literatures are case reports. Before a diagnosis is made, other primary and metastatic clear cell tumors need to be excluded. Immunohistochemistry is helpful for diagnosis and differential diagnosis. Surgical resection is the mainstay of treatment, and may be supplemented by radiotherapy.