鼻腔鼻窦转移性肾透明细胞癌一例报道并文献综述

doi:10.6040/j.issn.1673-3770.0.2021.065

摘要/Abstract

摘要： 目的探讨鼻腔鼻窦转移性肾透明细胞癌的临床特点及诊疗方法。方法回顾性分析1例肾透明细胞癌转移至鼻腔鼻窦患者病历资料,并检索1993~2020年间国内外发表的相关文献,包括本例共有64篇文献76例患者纳入研究,总结其临床表现、诊断、治疗方法及预后。结果 76例中男61例,女15例。症状出现较多者为鼻出血58例、鼻塞25例,受累部位多在鼻腔52例、筛窦41例、上颌窦22例。无肾癌病史28例,以鼻腔鼻窦转移灶为首发表现。既往有肾癌病史的患者发现鼻腔鼻窦转移灶距离原发灶时间为15 d~27年,平均间隔时间为46个月,中位间隔时间12个月。CT及MRI主要表现为鼻腔、鼻窦内软组织团块,注射造影剂后增强明显。确诊依据病理及免疫组化,容易误诊,76例中17例出现误诊,最常见误诊为血管瘤。76例中单纯手术治疗25例(32.9%),手术治疗结合放/化疗/靶向治疗的有28例(36.8%)。有随访资料记载的共51例,治疗后随访时间为1个月~14年,中位随访时间为12个月。手术可以明显改善患者预后(P<0.001),鼻腔鼻窦转移灶发现距原发灶时间>4个月患者生存率高于相隔时间≤4个月患者(P=0.008 7)。鼻腔鼻窦转移灶作为肾透明细胞癌首发部位与非首发部位(P=0.14)、单发转移灶与多发转移灶(P=0.10)、手术后放疗与无放疗(P=0.52)患者在生存率上差异无统计学意义。结论鼻腔鼻窦转移性肾透明细胞癌较为罕见,以中老年男性为主,可作为肾透明细胞癌首发部位,也可在原发灶治疗之后较长时间出现,主要临床表现为鼻出血及鼻塞,增强CT及MRI表现为肿物明显强化,明确诊断依靠病理及免疫组化,易误诊为血管瘤。治疗以手术切除为主的综合治疗,推荐血管栓塞后的鼻内镜手术,手术能改善患者预后,无法手术患者可采取靶向治疗或放化疗。

关键词: 肾透明细胞癌, 鼻腔, 鼻窦, 转移性, 血管瘤

Abstract: Objective To summarize and analyze the clinical features, diagnostic methods, treatment, and prognosis of patients with renal clear cell carcinoma(RCCC)that has metastasized to the nasal cavity and paranasal sinuses. Methods We conducted a retrospective review of the records of one patient with RCCC that had metastasized to the paranasal sinuses. We also performed a literature review and included 64 relevant studies published in Chinese and English from 1993 to 2020, including this case. A total of 76 patients with renal clear cell carcinoma that had metastasized to the paranasal sinuses were included, and their clinical manifestations, diagnosis, treatment methods, and prognosis were summarized. Results The 76 patients included 61 men and 15 women, with the most common symptoms being epistaxis in 58 patients and nasal obstruction in 25. The metastases were located in the nasal cavity(n=52), ethmoid sinus(n=41), and maxillary sinus(n=22). Twent-eight patients had sinonasal metastasis of renal clear cell carcinoma at first presentation. The delay between the diagnosis of the primary tumor and the diagnosis of metastasis to the sinonasal cavity ranged from 15 days to 27 years(mean, 46 months; median, 12 months). On CT and MRI, the lesions exhibited osteolysis and strong enhancement after contrast injection. The misdiagnosis rate was 22%. Immunohistochemistry was crucial for the correct diagnosis, and the most commonly used markers were CK, Vimentin, CD10, PAX8. Twenty-five patients(32.9%)underwent surgical management alone, 28 patients(36.8%)had surgical management combined with radiotherapy/chemotherapy/targeted therapy, 19 patients(25.0%)received targeted therapy alone and/or chemoradiotherapy, and 4 patients received no treatment. Follow-up data were available for 51 patients, and the duration of follow-up ranged from 1 month to 14 years(median, 12 months). There was a strong statistically significant association between surgery and a better prognosis(P<0.001). The overall survival was significantly better for patients whose duration between the primary tumor and metastasis was more than 4 months vs. no more than 4 months(P=0.008 7). There was no statistical difference in overall survival between patients who had sinonasal metastasis at first presentation of RCCC and those who had already been diagnosed with RCCC earlier(P=0.14), an isolated metastasis rather than multiple metastases(P=0.1), and surgery alone rather than surgery combined with radiotherapy(P=0.52). Conclusion Metastatic renal clear cell carcinoma to the nasal cavity and paranasal sinuses is rare. It mainly affects elderly males and can be diagnosed at the initial presentation of RCCC or long after treatment of the primary tumor. The most common symptoms are epistaxis and nasal obstruction. On CT and MRI, the lesions exhibit strong enhancement after contrast injection. The diagnosis depends on pathology and immunohistochemistry. It can easily be misdiagnosed as hemangioma. Surgical resection is the main treatment, and nasal endoscopic surgery after vascular embolization is recommended as it can improve the prognosis of the patients. Patients who cannot undergo surgery can undergo targeted therapy or chemoradiotherapy, with a poor overall prognosis.

Key words: Renal clear cell carcinoma, Nasal cavity, Paranasal sinuses, Metastases, Hemangioma

中图分类号:

R765

夏彩风,谢瑞玲,甄甄,文锋,王全桂. 鼻腔鼻窦转移性肾透明细胞癌一例报道并文献综述[J]. 山东大学耳鼻喉眼学报, 2021, 35(5): 36-45.

XIA Caifeng, XIE Ruiling, ZHEN Zhen, WEN Feng, WANG Quangui. Diagnosis and treatment of renal clear cell carcinoma that has metastasized to the nasal cavity and paranasal sinuses: a case report and systematic review[J]. Journal of Otolaryngology and Ophthalmology of Shandong University, 2021, 35(5): 36-45.

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