Adenoid cystic carcinoma (AdCC) is among the most common malignant tumors of the salivary glands. It is characterized by a prolonged clinical course, with frequent local recurrences, late onset of metastases and fatal outcome. High-grade transformation (HGT) is an uncommon phenomenon among salivary carcinomas and is associated with increased tumor aggressiveness. In AdCC with high-grade transformation (AdCC-HGT), the clinical course deviates from the natural history of AdCC. It tends to be accelerated, with a high propensity for lymph node metastasis. In order to shed light on this rare event and, in particular, on treatment implications, we undertook this review: searching for all published cases of AdCC-HGT. We conclude that it is mandatory to perform elective neck dissection in patients with AdCC-HGT, due to the high risk of lymph node metastases associated with transformation.

Coordinator of the International Head and Neck Scientific Group Padua Italy

Department of Anatomic Pathology Hospital Clinic University of Barcelona Barcelona Spain

Department of Biomedical Sciences and Medicine University of Algarve Faro Portugal

Department of Head and Neck Surgery and Otorhinolaryngology A C Camargo Cancer Center São Paulo Brazil

Department of Head and Neck Surgery Head and Neck Oncology Program St Luc University Hospital and King Albert 2 Cancer Institute Brussels Belgium

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

Department of Otolaryngology Head and Neck Surgery Neurological Institute of New Jersey Rutgers New Jersey Medical School Newark NJ USA

Department of Otolaryngology Head and Neck Surgery Nottingham University Hospitals Queens Medical Centre Campus Nottingham UK

Department of Otolaryngology Head and Neck Surgery Philipp University Marburg Germany

Department of Otolaryngology Head and Neck Surgery Radboud University Medical Center Nijmegen The Netherlands

Department of Otolaryngology Hospital Universitario Central de Asturias Oviedo Spain

Department of Otorhinolaryngology The University of Oklahoma Health Sciences Center Oklahoma City OK USA

Department of Pathology Allegiance Health Jackson MI USA

Department of Pathology Beth Israel Medical Center New York NY USA

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

Department of Pathology Radboud University Medical Center Nijmegen The Netherlands

Department of Pathology The University of Texas MD Anderson Cancer Center Houston TX USA

Department of Pathology University of Pittsburgh School of Medicine Pittsburgh PA USA

Department of Radiation Oncology Institute of Oncology Ljubljana Slovenia

Department of Radiation Oncology University of Florida Gainesville FL USA

Department of Surgery Banner MD Anderson Cancer Center Gilbert AZ USA

Departments of Pathology and Otolaryngology Head and Neck Surgery The Johns Hopkins Medical Institutions Baltimore MD USA

Departments of Surgery and Otolaryngology Head and Neck Surgery Albert Einstein College of Medicine Montefiore Medical Center Bronx NY USA

Division of Otolaryngology Head and Neck Surgery Southern Illinois University School of Medicine Springfield IL USA

European Salivary Gland Society Geneva Switzerland

Fundación de Investigación e Innovación Biosanitaria del Principado de Asturias Oviedo Spain

Head and Neck Surgery Memorial Sloan Kettering Cancer Center New York NY USA

Instituto Universitario de Oncología del Principado de Asturias University of Oviedo Oviedo Spain

Oral and Maxillofacial Pathology School of Dentistry University of Liverpool and Cellular Pathology Liverpool Clinical Laboratories Liverpool UK

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

Southern California Permanente Medical Group Woodland Hills CA USA

University of Udine School of Medicine Udine Italy

University Pathologists Fall River MA USA

University Pathologists Providence RI USA

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