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.

Department of Head and Neck Surgical Oncology University Medical Center Utrecht Utrecht The Netherlands

Department of Imaging and Pathology University Hospitals Leuven KU Leuven Leuven Belgium

Department of Oncology Section Head and Neck Oncology Otorhinolaryngology Head and Neck Surgery and Leuven Cancer Institute University Hospitals Leuven KU Leuven Leuven Belgium

Department of Otolaryngology Hospital Santa Creu i Sant Pau Barcelona Spain

Department of Otorhinolaryngology Head and Neck Surgery University of Helsinki and Helsinki University Hospital Helsinki Finland

Department of Otorhinolaryngology Institute of Phoniatry Pedaudiology Jena University Hospital Jena Germany

Department of Pathology and Genetics Sahlgrenska Cancer Center University of Gothenburg Gothenburg Sweden

Department of Pathology Charles University Faculty of Medicine in Plzen Plzen Czech Republic

Department of Pathology University of Arkansas for Medical Sciences Little Rock AR USA

Department of Pathology University of Texas Southwestern Medical Center Dallas TX USA

Department of Radiation Oncology Institute of Oncology Ljubljana Slovenia

Division of Ear Nose and Throat Diseases Department of Clinical Sciences Intervention and Technology Karolinska Institutet Karolinska University Hospital Stockholm Sweden

Epigenetics and Human Disease Laboratory CBMR Department of Biomedical Sciences and Medicine University of Algarve Faro Portugal

International Head and Neck Scientific Group Padua Italy

Multidisciplinary Salivary Gland Society Geneva Switzerland

Pathology Department Liverpool Clinical Laboratories Liverpool UK

School of Dentistry University of Liverpool Liverpool UK

University of Udine School of Medicine Udine Italy

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