Hellquist, HenrikSkalova, AlenaBarnes, LeonCardesa, AntonioThompson, Lester D. R.Triantafyllou, AsteriosWilliams, Michelle D.Devaney, Kenneth O.Gnepp, Douglas R.Bishop, Justin A.Wenig, Bruce M.Suarez, CarlosRodrigo, Juan P.Coca-Pelaz, AndresStrojan, PrimozShah, Jatin P.Hamoir, MarcBradley, Patrick J.Silver, Carl E.Slootweg, Pieter J.Vander Poorten, VincentTeymoortash, AfshinMedina, Jesus E.Robbins, K. ThomasPitman, Karen T.Kowalski, Luiz P.de Bree, RemcoMendenhall, William M.Eloy, Jean AndersonTakes, Robert P.Rinaldo, AlessandraFerlito, Alfio2017-04-072017-04-072016-070741-238Xhttp://hdl.handle.net/10400.1/9584Adenoid 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.engjournal article10.1007/s12325-016-0298-5