ABSTRACT
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.
Subject(s)
Ameloblastoma/diagnostic imaging , Choristoma/diagnostic imaging , Neoplasm Recurrence, Local/diagnosis , Odontogenic Tumors/diagnostic imaging , Paranasal Sinus Neoplasms , Ameloblastoma/genetics , Ameloblastoma/surgery , Choristoma/genetics , Choristoma/surgery , DNA Mutational Analysis , Fatal Outcome , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Nasal Cavity , Odontogenic Tumors/genetics , Odontogenic Tumors/surgery , Polymerase Chain Reaction , Proto-Oncogene Proteins B-raf/genetics , Tomography, X-Ray ComputedABSTRACT
AIM: The aim of this study was to evaluate almost 20 years of using the pectoralis major flap in head and neck reconstruction at the Department of Maxillofacial Surgery, Zagreb. PATIENTS: In the period from 1981 to 1999, a total of 506 pectoralis major flaps were used for head and neck reconstruction in 500 patients. In all cases the flap was used after surgical resection of an advanced malignant tumour of the head and neck. RESULTS: The tumours were intraoral in 387 cases (77%), pharyngeal in 78 cases (15%) and on the skin in 10 cases (5%). The defect was located in the mucosal lining in 407 (81%), skin in 43 (8%), both intra- and extraoral in 53 (10%) patients. Bone defects occurred in 65 patients. In 31 patients (6%), the pectoralis major flap was used in combination with other flaps (deltopectoral, tongue, trapezius and free flaps). Complications occurred with 168 flaps (33%), but total flap necrosis was only seen in 10 patients (2%). Surgical treatment of complications was necessary in 87 patients (17%). CONCLUSION: Despite the increasing use of microvascular reconstruction, the pectoralis major myocutaneous flap continues to be the most universal major flap in head and neck reconstruction.
