Vander Poorten Vincent, Triantafyllou Asterios, Skálová Alena, Stenman Göran, Bishop Justin A, Hauben Esther, Hunt Jennifer L, Hellquist Henrik, Feys Simon, De Bree Remco, Mäkitie Antti A, Quer Miquel, Strojan Primož, Guntinas-Lichius Orlando, Rinaldo Alessandra, Ferlito Alfio
Department of Oncology-Section Head and Neck Oncology, Otorhinolaryngology-Head and Neck Surgery and Leuven Cancer Institute, University Hospitals Leuven, KU Leuven, Leuven, Belgium.
Multidisciplinary Salivary Gland Society, Geneva, Switzerland.
Eur Arch Otorhinolaryngol. 2018 Jul;275(7):1681-1695. doi: 10.1007/s00405-018-4985-5. Epub 2018 May 14.
Although relatively rare, polymorphous adenocarcinoma (PAC) is likely the second most common malignancy of the minor salivary glands (MiSG). The diagnosis is mainly based on an incisional biopsy. The optimal treatment comprises wide surgical excision, often with adjuvant radiotherapy. In general, PAC has a good prognosis. Previously, PAC was referred to as polymorphous low-grade adenocarcinoma (PLGA), but the new WHO classification of salivary gland tumours has also included under the PAC subheading, the so-called cribriform adenocarcinoma of minor salivary glands (CAMSG). This approach raised controversy, predominantly because of possible differences in clinical behaviour. For example, PLGA (PAC, classical variant) only rarely metastasizes, whereas CAMSG often shows metastases to the neck lymph nodes. Given the controversy, this review reappraises the definition, epidemiology, clinical presentation, diagnostic work-up, genetics, treatment modalities, and prognosis of PAC of the salivary glands with a particular focus on contrasting differences with CAMSG.
多形性腺癌（PAC）虽然相对少见，但很可能是小涎腺（MiSG）的第二常见恶性肿瘤。诊断主要基于切开活检。最佳治疗方法包括广泛手术切除，通常还需辅助放疗。总体而言，PAC预后良好。以前，PAC被称为多形性低度腺癌（PLGA），但世界卫生组织（WHO）唾液腺肿瘤的新分类也将所谓的小涎腺筛状腺癌（CAMSG）归入PAC标题下。这种分类方法引发了争议，主要是因为临床行为可能存在差异。例如，PLGA（PAC，经典型）很少发生转移，而CAMSG常出现颈部淋巴结转移。鉴于存在争议，本综述重新评估了唾液腺PAC的定义、流行病学、临床表现、诊断检查、遗传学、治疗方式和预后，特别关注与CAMSG的对比差异。
