Yuan C C, Zhai C W, Wang S Y
Department of Pathology, Eye, Ear, Nose and Throat Hospital, Fudan University, Shanghai 200031, China.
Zhonghua Bing Li Xue Za Zhi. 2020 Nov 8;49(11):1147-1151. doi: 10.3760/cma.j.cn112151-20200206-00070.
To investigate the clinicopathological features, immunophenotype, differential diagnosis and prognosis of sinonasal renal cell-like adenocarcinoma. 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. 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. 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.
探讨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。对复旦大学附属眼耳鼻喉科医院2014年8月至2018年12月收治的鼻窦癌病例进行回顾性分析。筛选出肾细胞样腺癌进行临床病理特征分析,并复习相关文献。鼻窦癌460例,其中腺癌70例(15.2%),肾细胞样腺癌5例(1.1%)。男性4例,女性1例,平均年龄46.5岁(29 - 52岁)。主要临床表现为鼻塞和鼻出血。鼻内镜检查发现红色息肉样肿物。影像学检查显示鼻腔及筛窦肿物,侵犯周围结构并伴有骨质破坏。镜下,肿瘤细胞呈巢状、腺泡状和微囊状排列,其间有丰富的毛细血管,伴有出血。细胞胞质透明,核分级低,核仁不明显。部分区域肿瘤侵犯骨组织。免疫组化标记物CKpan、CK7、CAⅨ、S - 100和波形蛋白呈阳性,Ki - 67增殖指数低。RCC、CD10、PAX8、p63、SMA、HHF35、钙调蛋白、CD117、TTF - 1以及神经内分泌标记物Syn和CHG均为阴性。FISH检测未发现EWSR1和ETV6基因重排。5例患者初诊后均接受手术切除。1例患者二次复发后行手术切除并辅助放疗,1例患者术后接受放疗,1例患者复发后行手术切除,1例患者无复发,1例患者复发后接受放疗。至2019年12月,5例患者均无远处转移,无瘤生存。原发性鼻窦肾细胞样腺癌是低级别非肠型腺癌的一种特殊亚型,发病率低,生物学行为惰性。目前,大多数文献为病例报告。在做出诊断前,需要排除其他原发性和转移性透明细胞肿瘤。免疫组化有助于诊断和鉴别诊断。手术切除是主要治疗方法,可辅以放疗。