原发鼻腔鼻窦腺癌临床病理特征分析(附3例)

Analysis of clinicopathological features of primary sinonasal adenocarcinoma(3 cases)

目的:探讨原发性鼻腔鼻窦腺癌临床病理特征、诊断与鉴别诊断、治疗与预后。方法:回顾性分析2015-2022年我院确诊的3例原发鼻腔鼻窦腺癌的临床、影像学和免疫组织化学、分子病理学特点,并复习国内外相关文献。结果:3例患者均为男性,平均年龄68岁,均无木屑、皮革类长期接触史,组织学类型分别为肠型腺癌、高级别非肠型腺癌、低级别非肠型腺癌。临床表现均为鼻阻伴鼻出血,影像学检查无特异性,均未考虑癌。镜下肠型腺癌排列呈小管-腺样,部分呈乳头状、筛状,可见坏死,高级别非肠型腺癌呈明显的侵袭性生长,以实性增生为主,可见明显的核多形性和坏死,低级别非肠型腺癌呈特征性的复杂腺样生长模式并可见背靠背腺体,肿瘤细胞一致,核圆形。分子病理检测:均未检测到BRAF基因突变。治疗方案为手术或手术+放/化疗,均随访17~36月,肠型腺癌和高级别非肠型腺癌患者术后均死亡,低级别非肠型腺癌患者无复发或转移。结论:原发鼻腔鼻窦腺癌罕见,无特异临床表现及影像学表现,诊断主要依赖病理组织学检查,免疫组化及分子病理可协助诊断,并对治疗和预后监测有指导作用。

Objective:To investigate the clinicopathological features,diagnosis and differential diagnosis,treatment and prognosis of primary sinonasal adenocarcinoma.Methods:The clinical data,imaging findings,pathological characteristics of three cases of primary sinonasal adenocarcinoma which were diagnosed in our hospital from 2015 to 2022 were collected and analyzed,and relevant domestic and foreign literatures were reviewed.Results:There were 3 males with an average age of 68 years.And none had a long-term exposure to wood dust or leather dust.The histological types were respectively intestinal-type adenocarcinoma(ITAC),high-grade non-intestinal-type adenocarcinoma(non-ITAC),and low-grade non-intestinal-type adenocarcinoma.They had similar clinical symptoms of the nasal obstruction and epistaxis.There was no specific evidence for cancer in their CT and MR imaging findings.Microscopically,the ITAC showed prominent papillary fronds with tubular-glandular pattern.And necrosis was visible.The high-grade non-ITAC showed a distinctly invasive growth pattern with solid hyperplasia and obvious nuclear pleomorphism.Necrosis was visible,too.While the low-grade non-ITAC showed a characteristic complex glandular growth pattern with back-to-back glands.The tumour cells are uniform with regular rounded nuclei.Molecular pathology:The BRAF mutation was not detected in the three cases.All the three patients accepted complete surgical resection or surgical resection with postoperative radiotherapy/chemotherapy management.The patient with ITAC and the another patient with high-grade non-ITAC both died several months later,while the patient with low-grade non-ITAC had no recurrence or metastasis.Conclusion:Primary sinonasal adenocarcinoma is rare.It has no specific clinical or imaging manifestations.And the diagnosis relies mainly on histology.Immunohistochemistry and molecular pathology can assist in the diagnosis and provide guidance for treatment and prognostic monitoring.