- AJSP 多中心病例系列（6 家三级中心，12 例），描述极为罕见的 HPV 相关口咽非涎腺型腺癌。
- 临床：有性别信息者均为男性，中位年龄 62 岁；原发多在腭扁桃体/扁桃体–腭区，常为 T3/T4。
- 病理要点：高级别腺癌形态、无可辨认鳞状成分；弥漫 p16+，PCR 或 ISH 证实高危 HPV；多数病例肿瘤细胞 p40 阴性（个别仅腔面外细胞 p40/CK5/6+）。
- 鉴别：须排除涎腺型腺癌与转移性腺癌，并确认 HPV 关联。随访有限，但可见远处转移与病死。原文为 Ovid/LWW 订阅内容，本站仅收摘要。
收录范围：PubMed 摘要中英对照。原文为 Ovid/LWW 订阅内容（licenseInfoList 为空），本站未获全文与图表。
摘要
人乳头瘤病毒（HPV）相关的口咽非涎腺型腺癌极为罕见，定义尚不清晰。本文报告来自 6 家三级中心的多机构系列，共 12 例，复习组织学、免疫组化、HPV 检测、分子及随访资料。11 例有性别信息者均为男性；中位年龄 62 岁（范围 46–70 岁）。队列包括 9 例原发性口咽腺癌与 3 例转移性腺癌（含 1 例推定口咽来源的右侧颈部转移，以及 2 例既往已确诊 HPV 相关口咽鳞癌后出现的远处转移）。原发部位：腭扁桃体或扁桃体–腭区（n=7）、舌根（n=1）、口咽未特指（n=1）。有 T 分期信息的病例中，6 例里 5 例为 T3/T4。所有肿瘤均呈高级别腺癌形态，无可辨认的鳞状成分，架构为浸润性腺样、导管样或筛状。全部病例呈弥漫 p16 表达，高危型 HPV 经聚合酶链反应或原位杂交证实。病例 1–6、8、9、11、12 的肿瘤细胞群 p40 阴性；病例 7 仅在腔面外（abluminal）细胞表达 p40 与 CK5/6。2 例分子谱检出累及 PIK3CA 与 PIK3R1 的改变或变异。6 例原发肿瘤有随访：3 例出现远处转移，1 例死于本病。HPV 相关口咽非涎腺型腺癌是一种诊断具有挑战性的肿瘤，扩展了 HPV 相关口咽癌的形态谱。识别时需排除涎腺型与转移性腺癌，并确认与 HPV 的关联。
- AJSP multi-institutional series (12 cases, 6 tertiary centers) of exceptionally rare HPV-associated non-salivary oropharyngeal adenocarcinoma.
- Clinical: all patients with known sex were men; median age 62 years; primaries often tonsil/tonsil-palate, frequently T3/T4.
- Pathology: high-grade adenocarcinoma without squamous component; diffuse p16 and high-risk HPV by PCR or ISH; tumor cells usually p40-negative (one case with abluminal-only p40/CK5/6).
- Differential: exclude salivary-type and metastatic adenocarcinoma and confirm HPV association. Limited follow-up includes distant metastases and disease-specific death. Ovid/LWW subscription — abstract only.
Scope: the PubMed abstract. The article is Ovid/LWW subscription content (empty licenseInfoList); the full text and figures were not obtained.
Abstract
Human papillomavirus (HPV)-associated non-salivary adenocarcinoma of the oropharynx is exceptionally rare and poorly defined. We report a multi-institutional series of 12 cases from 6 tertiary centers. Histologic, immunohistochemical, HPV testing, molecular, and follow-up data were reviewed. Among 11 patients with available sex data, all were men; the median age was 62 years (range: 46 to 70 y). The cohort comprised 9 primary oropharyngeal adenocarcinomas and 3 metastatic adenocarcinomas, including a right neck metastasis of presumed oropharyngeal origin and 2 distant metastases in patients with previously documented HPV-associated oropharyngeal squamous cell carcinoma. Primary sites included the palatine tonsil or tonsil-palate region (n=7), base of tongue (n=1), and oropharynx, not otherwise specified (n=1). Among cases with available T-category, 5 of 6 were T3/T4. All tumors showed high-grade adenocarcinoma morphology without a recognizable squamous component, with infiltrative glandular, ductal, or cribriform architecture. Diffuse p16 expression was present in all cases, and high-risk HPV was confirmed by polymerase chain reaction or in situ hybridization. p40 was negative in the tumor cell population in cases 1 to 6, 8, 9, 11, and 12, whereas case 7 showed p40 and CK5/6 expression confined to abluminal cells. Molecular profiling in 2 cases identified alterations or variants involving PIK3CA and PIK3R1. Follow-up in 6 patients with primary tumors showed distant metastases in 3 and disease-specific death in 1. HPV-associated nonsalivary adenocarcinoma of the oropharynx is a diagnostically challenging tumor that expands the morphologic spectrum of HPV-related oropharyngeal carcinoma. Recognition requires exclusion of salivary-type and metastatic adenocarcinoma and confirmation of HPV association.