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神经纤维瘤病 1 型合并下颌骨双侧中央性巨细胞肉芽肿。

Neurofibromatosis type 1 associated with bilateral central giant cell granuloma of the mandible.

机构信息

Department of Oral and Maxillofacial Surgery, School of Dentistry, Pontifícia Universidade Católica de Minas Gerais, Av. Dom José Gaspar, 500 Prédio 45, Coração Eucarístico, Belo Horizonte, MG, Brazil.

出版信息

J Craniomaxillofac Surg. 2011 Oct;39(7):538-43. doi: 10.1016/j.jcms.2010.10.014. Epub 2010 Nov 11.

DOI:10.1016/j.jcms.2010.10.014
PMID:21071237
Abstract

Neurofibromatosis type 1, or von Recklinghausen disease, is one of the most common hereditary neurocutaneous disorders in humans. Clinically, Neurofibromatosis type 1 is characterized by café-au-lait spots, freckling, skin neurofibroma, plexiform neurofibroma, bony defects, Lisch nodules and tumors of the central nervous system. Central giant cell granuloma is a benign central lesion of bone, primarily involving the jaws, of variably aggressive nature characterized by aggregates of multinucleated giant cells in a background of cellular vascular fibrous connective tissue and spindle-shaped mononuclear stromal cells. The association between neurofibromatosis and central giant cell granuloma has been reported in the literature. A case of mandibular bilateral central giant cell granuloma in a patient with Neurofibromatosis type 1 was conservatively but successfully treated by adequate surgical curettage of mandibular bone lesions.

摘要

神经纤维瘤病 1 型,又称 von Recklinghausen 病,是人类最常见的遗传性神经皮肤疾病之一。临床上,神经纤维瘤病 1 型的特征是咖啡牛奶斑、雀斑、皮肤神经纤维瘤、丛状神经纤维瘤、骨缺损、神经节细胞瘤和中枢神经系统肿瘤。颌骨中央性巨细胞瘤是一种良性的骨中央病变,主要累及颌骨,其性质具有不同程度的侵袭性,特征是多核巨细胞聚集在细胞血管纤维结缔组织和梭形单核基质细胞的背景中。神经纤维瘤病与颌骨中央性巨细胞瘤之间的关联在文献中有报道。本文报道了 1 例神经纤维瘤病 1 型患者双侧下颌骨中央性巨细胞瘤,通过充分的下颌骨病变刮除术进行保守但成功的治疗。

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