Department of Pathology and Laboratory Medicine, Rutgers Robert Wood Johnson Medical School, One Robert Wood Johnson Place, New Brunswick, NJ, 08903, USA.
Department of Pathology and Laboratory Medicine, Drexel University/Hahnemann University Hospital, 245 N. 15th St., Philadelphia, PA, 19102-1192, USA.
Head Neck Pathol. 2020 Jun;14(2):473-479. doi: 10.1007/s12105-019-01069-3. Epub 2019 Aug 31.
Human papillomavirus (HPV)-related multiphenotypic sinonasal carcinoma is a distinct, recently described neoplasm of salivary gland nature that has an unusual microscopic appearance exhibiting multidirectional differentiation. Originally described by Bishop et al. in 2012, this distinct form of head and neck cancer is a very rare entity that few pathologists have encountered in practice, and only 50 cases have been reported in the literature. It usually presents as a large, destructive mass confined to the nasal cavity or paranasal sinuses, and is always associated with high-risk HPV infection. Although histologically it often resembles adenoid cystic carcinoma, this neoplasm also consistently exhibits features of myoepithelial, ductal and squamous differentiation. Newly recognized characteristics have recently been described that include bizarre pleomorphism, sarcomatoid transformation, and heterologous cartilaginous differentiation. These unique features have continued to expand the morphologic spectrum of this neoplasm and justify the recent change in its nomenclature from "HPV-related carcinoma with adenoid cystic-like features" to "HPV-related multiphenotypic sinonasal carcinoma (HMSC)". In 2017, "HPV-related carcinoma with adenoid cystic like features" was included as a provisional tumor type by the World Health Organization Classification of Head and Neck Tumors. Despite the presence of high-grade histologic characteristics such as necrosis and brisk mitotic activity, and a tendency for recurrence, HMSC demonstrates indolent clinical behavior and carries a good prognosis.
人乳头瘤病毒（HPV）相关的多表型鼻旁窦癌是一种独特的、最近描述的具有唾液腺性质的肿瘤，其具有不寻常的微观外观，表现出多向分化。这种独特的头颈部癌症最初由 Bishop 等人于 2012 年描述，是一种非常罕见的实体，很少有病理学家在实践中遇到，文献中仅报道了 50 例。它通常表现为局限于鼻腔或副鼻窦的大而破坏性肿块，始终与高危 HPV 感染相关。尽管组织学上它常类似于腺样囊性癌，但这种肿瘤还始终表现出肌上皮、导管和鳞状分化的特征。最近描述了一些新的特征，包括奇异的多形性、肉瘤样转化和异源性软骨分化。这些独特的特征不断扩大了这种肿瘤的形态谱，并证明了其最近在命名上的变化，从“具有腺样囊性特征的 HPV 相关癌”改为“HPV 相关的多表型鼻旁窦癌（HMSC）”。2017 年，“具有腺样囊性样特征的 HPV 相关癌”被世界卫生组织头颈部肿瘤分类列为暂定肿瘤类型。尽管存在高级别的组织学特征，如坏死和活跃的有丝分裂活动，以及复发的倾向，但 HMSC 表现出惰性的临床行为，并具有良好的预后。
