Ameloblastic Fibrosarcoma Involving the Anterior and Middle Skull Base With Intradural Extension
Ameloblastic Fibrosarcoma Involving the Anterior and Middle Skull Base With Intradural Extension
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累及前颅底和中颅底并伴有硬膜内扩展的成釉细胞纤维肉瘤
DOI：
10.1097/scs.0b013e3181c50cf7
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发表时间：
2009
影响因子：
0.9
通讯作者：
S. Prabhu
中科院分区：
文献类型：
--
作者：
Bharat Guthikonda;E. Hanna;R. Skoracki;S. Prabhu
成釉细胞纤维肉瘤是一种恶性牙源性肿瘤，很少影响颅底和周围地区。我们报告一位48岁男性，在初次出现面部肿块10年后，经组织学证实为良性造釉细胞纤维瘤恶变。成釉细胞纤维肉瘤从面部向眼眶、前颅窝和中颅窝颅底、颞下窝和海绵窦延伸。进行性眼球突出，单眼视力完全丧失为主要症状。根据我们的回顾，我们的病例是第一例成釉细胞纤维肉瘤硬膜内扩散的报告。采用多学科颅底入路，切除除海绵窦内肿瘤外的所有肿瘤，并用股前外侧游离皮瓣重建缺损。患者术后没有新的神经功能缺损，并接受了辅助分次放疗。成釉细胞纤维瘤恶性转化为成釉细胞纤维肉瘤可发生在最初的表现多年后。因此，尽管最初的病理性病变是良性的，但警惕的长期随访是必要的。在这些复杂的病例中，使用多学科方法对于获得最佳结果至关重要。
Ameloblastic fibrosarcoma is a malignant odontogenic tumor that rarely affects the skull base and surrounding regions. We present a case of a 48-year-old man with histologically confirmed malignant transformation of a benign ameloblastic fibroma 10 years after initial presentation of a localized facial mass. The ameloblastic fibrosarcoma extended from the facial region to the orbit, anterior and middle fossa skull base, the infratemporal fossa, and the cavernous sinus. Progressive proptosis with complete monocular vision loss was the presenting symptom. To our review, our case represents the first report of intradural extension of ameloblastic fibrosarcoma. Using a multidisciplinary skull base approach, resection of all tumors except that in the cavernous sinus was achieved with the resulting defect reconstructed with an anterolateral thigh free flap. The patient had no new neurologic deficits after surgery and underwent adjuvant fractionated radiation therapy. Malignant transformation of ameloblastic fibroma into ameloblastic fibrosarcoma can occur many years after initial presentation. Thus, vigilant long-term follow-up is essential despite the benign nature of the initial pathologic lesion. Use of a multidisciplinary approach is critical in obtaining the optimal outcome in these complex cases.