Tarle Marko, Müller Danko, Tarle Antonia, Blivajs Igor, Aljinović Ratković Naranđa, Knežević Predrag
Marko Tarle, Department of Oral and Maxillofacial Surgery, University Hospital Dubrava, Gojko Šušak Avenue 6, 10000 Zagreb, Croatia,
Croat Med J. 2020 Jul 5;61(3):271-275. doi: 10.3325/cmj.2020.61.271.
Ameloblastic 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.
成釉细胞癌（AC）是一种罕见的侵袭性恶性上皮性牙源性肿瘤，最常见于下颌骨或上颌骨。迄今为止，异位成釉细胞癌（EAC）作为AC一种极其罕见的颌骨外定位且与颌骨无关联的情况，仅被描述过三次。本报告介绍了一名患有颅底成釉细胞癌的64岁男性，并对与EAC相关的诊断和治疗挑战进行了综述。由于其罕见性以及与其他肿瘤在组织学上的相似性，EAC常被误诊。这就是我们建立EAC的病理组织学和免疫组化特征以将其与组织学上相似肿瘤区分开来的原因。EAC最常用的治疗方法是根治性手术切除，但大多数综述报告描述了局部复发情况。考虑到关于成釉细胞瘤分子发病机制的新科学发现，我们是首个对一名EAC患者进行BRAF突变分析的。BRAF抑制剂在BRAF阳性成釉细胞瘤的治疗中显示出有前景的结果，应继续在AC和EAC患者中进行研究。最后，在颌骨外头颈部肿瘤的鉴别诊断中应考虑EAC。
