• This ESMO–EURACAN Clinical Practice Guideline provides key recommendations for managing salivary gland cancer. • The guideline covers clinical and pathological diagnosis, staging and risk assessment, treatment and follow-up. • Treatment algorithms for parotid, submandibular, sublingual and minor salivary gland cancer are provided. • The author group encompasses a multidisciplinary group of experts from different institutions and countries in Europe. • Recommendations are based on available scientific data and the authors’ collective expert opinion.

• Keywords
• ESMO–EURACAN Clinical Practice Guideline, diagnosis, follow-up, salivary gland cancer, treatment,
• MeSH
• Adult MeSH
• Medical Oncology MeSH
• Humans MeSH
• Salivary Gland Neoplasms * diagnosis   therapy   MeSH
• Follow-Up Studies MeSH
• Check Tag
• Adult MeSH
• Humans MeSH
• Publication type
• Journal Article MeSH
• Practice Guideline MeSH
• Geographicals
• Europe MeSH

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.

• Keywords
• Cribriform adenocarcinoma of minor salivary glands, Pathology, Polymorphous adenocarcinoma, Polymorphous low-grade adenocarcinoma, Prognosis, Salivary glands, Therapy,
• MeSH
• Adenocarcinoma pathology   surgery   therapy   MeSH
• Humans MeSH
• Salivary Glands, Minor * MeSH
• Salivary Gland Neoplasms pathology   therapy   MeSH
• Prognosis MeSH
• Check Tag
• Humans MeSH
• Publication type
• Journal Article MeSH
• Review MeSH