成人鼻咽血管纤维瘤：按年龄分层的分期分布、临床结局及探索性肿瘤-正常基因组学
Nasopharyngeal Angiofibroma in Adults: Age-Stratified Stage Distribution, Clinical Outcomes, and Exploratory Tumor-Normal Genomics.
文献信息
| PMID | 42882874 |
|---|---|
| 原文 | 在 PubMed 查看原文 ↗ |
| 发表日期 | 2026 |
| 作者 | Haoyuan Xu |
| 作者单位 | Department of Otolaryngology-Head and Neck Surgery, Eye & ENT Hospital of Fudan University, 83 Fenyang Road, Shanghai 200031, China. |
| 期刊 | Cancers |
| SCI 分区 | Q2 |
| IF | 5.1 |
| 研究类型 | 临床研究 · 临床 |
| 所属专科 | 鼻科 |
中文摘要
背景/目的： 鼻咽血管纤维瘤（NA）通常为良性，但复发疾病及罕见的恶性转化在青春期之后仍具有临床意义。我们考察了成人临床表现、恶性转化记录以及分期分布的年龄相关差异。
方法： 我们回顾性复习了2006年1月至2026年6月期间接受手术治疗的289例患者。使用精确检验比较年龄>30岁与≤30岁患者之间的Radkowski分期分布。对16例成人复习了详细临床记录，并对一对肿瘤-血液样本检查了全基因组测序（WGS）注释摘要。
结果： 25例患者（8.7%）年龄大于30岁；287例患者有可用的分期。在详细成人亚组中，一份既往病理切片记录描述了一例复发肿瘤存在局灶性恶性转化，Ki-67指数约为50%。另有2例患者在数据库中有转化条目，但无 accompanying 病理学记录。记录了4例复发和3例死亡，均发生于IIIB期疾病。3名男性有结直肠癌或直肠癌病史。低、中、晚期肿瘤分别占成人病例的36.0%、36.0%和28.0%，而较年轻病例分别为17.6%、57.3%和25.2%（p = 0.0493）。基因组注释显示以C>A为主的替换谱。
结论： 成人NA包括复发性IIIB期疾病以及一例有记录的局灶性恶性转化。不同年龄组之间的分期分布存在差异。这些临床观察支持对复发肿瘤进行病理学再评估。单例基因组发现仍属描述性注释。
英文摘要
BACKGROUND/OBJECTIVES: Nasopharyngeal angiofibroma (NA) is generally benign, but recurrent disease and rare malignant transformation remain clinically relevant beyond adolescence. We examined adult clinical presentations, records of malignant transformation, and age-related differences in stage distribution.
METHODS: We retrospectively reviewed 289 surgically treated patients from January 2006 through June 2026. Radkowski stage distributions were compared between patients aged > 30 and ≤30 years using exact tests. Detailed clinical records were reviewed for 16 adults, and whole-genome sequencing (WGS) annotation summaries were examined for one tumor-blood pair.
RESULTS: Twenty-five patients (8.7%) were older than 30 years; stage was available for 287 patients. In the detailed adult subgroup, a prior pathology-slide record described focal malignant transformation with a Ki-67 index of approximately 50% in one recurrent tumor. Two other patients had database transformation entries without accompanying pathological documentation. Four recurrences and three deaths were recorded, all in stage IIIB disease. Three men had colorectal or rectal cancer histories. Low-, intermediate-, and advanced-stage tumors accounted for 36.0%, 36.0%, and 28.0% of adult cases, compared with 17.6%, 57.3%, and 25.2% of younger cases (p = 0.0493). The genomic annotations showed a C>A-predominant substitution spectrum.
CONCLUSIONS: Adult NA included recurrent stage IIIB disease and a documented record of focal malignant transformation. Stage distributions differed between age groups. These clinical observations support pathological reassessment of recurrent tumors. The single-case genomic findings remain descriptive annotations.