• Marko Tarle, Danko Müller, Antonia Tarle, Igor Blivajs, Naranđa Aljinović Ratković, and Predrag Knežević.
• Marko Tarle, Department of Oral and Maxillofacial Surgery, University Hospital Dubrava, Gojko Šušak Avenue 6, 10000 Zagreb, Croatia, tarlemarko1@gmail.com.
• Croat. Med. J. 2020 Jul 5; 61 (3): 271-275.

AbstractAmeloblastic carcinoma (AC) is a rare and aggressive malignant epithelial odontogenic tumor, most commonly located in the mandible or maxilla. An extremely rare extragnathic localization of AC with no connection to the jaws, ectopic ameloblastic carcinoma (EAC), has so far been described only three times. This report presents a 64-year-old male with skull base ameloblastic carcinoma and offers a review of diagnostic and treatment challenges related to EAC. Because of its rarity and histological similarity to other tumors, EAC is often misdiagnosed. This is why we established a pathohistological and immunohistochemical profile of EAC that differentiates it from histologically similar tumors. The most frequently used EAC treatment is radical surgical resection, but the majority of reviewed reports described local recurrence. Taking into consideration new scientific discoveries on the molecular pathogenesis of ameloblastoma, we are the first to have performed BRAF mutation analysis in an EAC patient. BRAF inhibitors offer promising results in the treatment of BRAF-positive ameloblastomas and should continue to be researched in AC and EAC patients. Finally, EAC should be considered in differential diagnosis of head and neck tumors outside the jaws.

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