Department of Pathology, Eye & ENT Hospital, Fudan University, Shanghai, People's Republic of China.
Am J Surg Pathol. 2023 Oct 1;47(10):1168-1175. doi: 10.1097/PAS.0000000000002092. Epub 2023 Jun 28.
The aim of this study was to evaluate the clinicopathologic features, molecular characteristics, treatment strategy, and prognosis of nasopharyngeal hyalinizing clear cell carcinoma (HCCC). Retrospective observational case series. Institutional pathology records between 2006 and 2022 were searched for all cases of nasopharyngeal HCCC. We included 10 male and 16 female patients aged 30 to 82 years (median: 60.5 y, mean: 54.6 y). The most common symptoms were blood-stained rhinorrhea and nasal obstruction. Tumors most often involved the lateral wall of the nasopharynx, followed by the superior posterior wall. Microscopically, all tumor cells were arranged in sheets, nests, cords, and single cells in a hyaline/myxoid/fibrous stroma. The tumor cells were polygonal, with or without distinct cell borders, and displayed abundant clear-to-eosinophilic cytoplasm. All 26 cases were positive for pancytokeratin, CK7, p40, and p63 but negative for myoepithelial differentiation markers. Ki-67 labeling was low and ranged from 1% to 10%. All 26 cases demonstrated EWSR1 and EWSR1-ATF1 rearrangements, and no case demonstrated MAML2 rearrangement. Complete follow-up data were available for 23 patients: 14 patients underwent endoscopic surgery alone, 5 underwent radiation therapy followed by endoscopic surgery, 3 underwent radiation therapy followed by biopsy, and 1 underwent cisplatin chemotherapy before endoscopic surgery. Clinical follow-up ranged from 6 to 195 months; 13 patients (56.5%) were alive without tumor, 5 patients (21.7%) died of disease, 5 patients (21.7%) survived with tumor. HCCCs of the nasopharynx are rare tumors. The definitive diagnosis depends on histopathology, immunohistochemistry, and molecular studies. The optimal treatment for patients with nasopharyngeal HCCC is wide local excision. Radiation and chemotherapy might be good options for managing locally advanced cases. Nasopharyngeal HCCC is less indolent than previously thought. Tumor stage and the choice of treatment are key factors affecting the prognosis of nasopharyngeal HCCC patients.
本研究旨在评估鼻咽透明细胞癌（HCCC）的临床病理特征、分子特征、治疗策略和预后。回顾性观察性病例系列。在 2006 年至 2022 年期间，通过机构病理学记录搜索所有鼻咽 HCCC 病例。我们纳入了 10 名男性和 16 名女性患者，年龄 30 至 82 岁（中位数：60.5 岁，均值：54.6 岁）。最常见的症状是血性鼻涕和鼻塞。肿瘤最常累及鼻咽侧壁，其次是后上壁。镜下，所有肿瘤细胞均呈片状、巢状、条索状和单个细胞排列在透明/黏液样/纤维基质中。肿瘤细胞呈多边形，有或无明显的细胞边界，显示丰富的透明至嗜酸性细胞质。所有 26 例均对广谱细胞角蛋白、CK7、p40 和 p63 阳性，但对肌上皮分化标志物阴性。Ki-67 标记物低，范围为 1%至 10%。所有 26 例均显示 EWSR1 和 EWSR1-ATF1 重排，无一例显示 MAML2 重排。23 例患者可获得完整的随访数据：14 例患者仅行内镜手术，5 例患者行放疗后内镜手术，3 例患者行放疗后活检，1 例患者在行内镜手术前行顺铂化疗。临床随访时间为 6 至 195 个月；13 例（56.5%）患者无肿瘤存活，5 例（21.7%）患者死于疾病，5 例（21.7%）患者存活带瘤。鼻咽透明细胞癌是一种罕见的肿瘤。明确诊断依赖于组织病理学、免疫组织化学和分子研究。患者的最佳治疗方法是广泛局部切除。放疗和化疗可能是治疗局部晚期病例的良好选择。鼻咽透明细胞癌的惰性较之前认为的要差。肿瘤分期和治疗选择是影响鼻咽透明细胞癌患者预后的关键因素。
