Andreasen Simon, Bishop Justin A, Hansen Thomas van Overeem, Westra William H, Bilde Anders, von Buchwald Christian, Kiss Katalin
Department of Otorhinolaryngology, Head and Neck Surgery and Audiology, Copenhagen University Hospital, Copenhagen, Denmark.
Department of Otorhinolaryngology and Maxillofacial Surgery, Zealand University Hospital, Køge, Denmark.
Histopathology. 2017 May;70(6):880-888. doi: 10.1111/his.13162. Epub 2017 Feb 24.
Human papillomavirus (HPV) is known as causative for squamous cell carcinoma (SCC) of the oropharynx, but is also found not infrequently in carcinomas of the sinonasal tract. Recently, a subset of these carcinomas was recognized to harbour HPV33 and have a significant morphological overlap with adenoid cystic carcinoma (ACC), a rare and aggressive carcinoma originating in the minor salivary glands. Termed 'HPV-related carcinoma with ACC-like features', only nine cases have been reported. To clarify the occurrence of these tumours we screened a large material for the presence of HPV-related ACC-like carcinoma. The identified tumours were characterized immunohistochemically and with fluorescence in-situ hybridization, and clinicopathological information for all cases is presented.
Forty-seven candidate cases were screened for presence of HPV. Six cases were identified and genotyped as HPV types 33, 35, and 56. All six cases had areas of dysplastic mucosal lining and showed remarkable heterogeneous morphologies. MYB, MYBL1, and NFIB genes were intact and, interestingly, staining for MYB protein was largely negative in contrast to what was found in ACC. One patient experienced a local recurrence 11 years after initial treatment and the remaining five patients were alive without evidence of disease.
We report six new cases of HPV-related ACC-like carcinoma and found that, although in a small material, the prognosis for these patients seems more favourable than for ACC. For the distinction between ACC and HPV-related ACC-like carcinoma, p16, MYB immunohistochemistry or investigation of MYB, MYBL1 and NFIB gene status are valuable.
人乳头瘤病毒（HPV）被认为是口咽鳞状细胞癌（SCC）的病因，但在鼻窦道癌中也并非罕见。最近，人们认识到这些癌中的一部分含有HPV33，并且与腺样囊性癌（ACC）有显著的形态学重叠，腺样囊性癌是一种起源于小唾液腺的罕见且侵袭性强的癌。这种被称为“具有腺样囊性癌样特征的HPV相关癌”的病例仅报告了9例。为了阐明这些肿瘤的发生情况，我们在大量样本中筛查了是否存在HPV相关的腺样囊性癌样癌。对鉴定出的肿瘤进行了免疫组织化学和荧光原位杂交特征分析，并给出了所有病例的临床病理信息。
对47例候选病例进行了HPV检测。鉴定出6例，基因分型为HPV 33型、35型和56型。所有6例均有发育异常的黏膜内衬区域，且形态显著异质性。MYB、MYBL1和NFIB基因完整，有趣的是，与腺样囊性癌中发现的情况相反，MYB蛋白染色大多为阴性。1例患者在初始治疗11年后出现局部复发，其余5例患者存活且无疾病证据。
我们报告了6例新的HPV相关腺样囊性癌样癌病例，发现尽管样本量小，但这些患者的预后似乎比腺样囊性癌更有利。对于腺样囊性癌和HPV相关腺样囊性癌样癌的鉴别，p16、MYB免疫组织化学或MYB、MYBL1和NFIB基因状态的检测很有价值。
