PDF(Pubmed)
Abstract：
Ameloblastic fibrosarcoma (AFS) is an extremely rare malignant odontogenic tumor. It is composed of benign odontogenic epithelium, resembling that of ameloblastoma, and a mesenchymal part exhibiting features of fibrosarcoma. The development of this lesion in the jaws is either de novo or from preexisting ameloblastic fibroma which has been well documented. The most commonly affected site within the jaw is the posterior mandible. These tumors show local aggressiveness and a high tendency to recur. We present a case of a 33-year-old female patient with swelling of the right posterior mandible for 2 months and progressive paresthesia of the same region for the past 6 months. Patient\'s history revealed undergoing surgical enucleation for ameloblastic fibroma before a year in the same region as current swelling. Examination of the swelling revealed an ulceroprolifeartive Growth of 6 × 4.5 cm extending from premolar to molar region. Primary investigation involved biopsy of the swelling, which was reported as sarcoma for which resection of the right hemimandible and selective neck dissection was performed. Following surgery, the final histopathology report of the resected specimen was reported to be AFS. One year after the surgical procedure, the patient is clinically and radiologically disease-free. Considering the aggressive nature of these tumors, it is vital to give an accurate diagnosis through biopsy, which is considered as gold standard diagnostic evidence, so that the surgeon plans the appropriate therapeutic decision. Knowledge of this rare entity and its histologic features as opposed to the more common benign counterparts such as ameloblastoma or ameloblastic fibroma is crucial as the latter involves a conservative treatment approach while the former can only be treated through aggressive resections.
摘要：
成釉细胞纤维肉瘤（AFS）是一种极为罕见的恶性牙源性肿瘤。它由良性牙源性上皮组成，类似成釉细胞瘤，和表现出纤维肉瘤特征的间质部分。颌骨中这种病变的发展要么是从头发生的，要么是由已有文献记载的成釉细胞纤维瘤引起的。颌内最常见的受影响部位是后下颌骨。这些肿瘤显示出局部侵袭性和高复发倾向。我们介绍了一例33岁的女性患者，其右后下颌骨肿胀2个月，并且在过去6个月中同一区域进行性感觉异常。患者的病史显示，在与当前肿胀相同的区域，一年前接受了成釉细胞纤维瘤手术摘除术。对肿胀的检查显示，从前磨牙到磨牙区域的溃疡生长为6×4.5cm。初步调查涉及肿胀的活检，据报道，这是肉瘤，对其进行了右半可食性切除术和选择性颈清扫术。手术后,切除标本的最终组织病理学报告为AFS.手术一年后,患者在临床和放射学上无疾病。考虑到这些肿瘤的侵袭性，通过活检进行准确的诊断至关重要，这被认为是黄金标准的诊断证据，以便外科医生计划适当的治疗决定。与成釉细胞瘤或成釉细胞纤维瘤等更常见的良性对应物相比，了解这种罕见实体及其组织学特征至关重要，因为后者涉及保守治疗方法，而前者只能通过积极切除治疗。
