Kubota Seiko, Imai Tomoaki, Iwai Soichi, Nakazawa Mitsuhiro, Uzawa Narikazu
Department of Oral and Maxillofacial Surgery II, Osaka University Graduate School of Dentistry, Osaka, Japan.
J Craniofac Surg. 2019 May/Jun;30(3):e205-e207. doi: 10.1097/SCS.0000000000005094.
Gingival enlargement, although frequently encountered in clinical settings, is rarely associated with systemic diseases or syndromes. Among the diverse pathological conditions of neurofibromatosis type 1 (NF-1), minor manifestations in the orofacial region are occasionally overlooked. Herein, the authors present an unusual case of gingival neurofibroma in a patient with NF-1 associated with characteristic osseous defects in the alveolus in the long-term course of 17 years from the first examination.A 5-year-old boy with NF-1 was referred for the evaluation of gingival enlargement in the posterior left maxilla. An incisal biopsy led to the diagnosis of neurofibroma. At 22 years of age, the patient was referred again with a complaint of bleeding and pain in the same region refractory to periodontal therapy. The gingiva and tuberosity were swollen, and the second molar was affected by the tumor. Radiography revealed a low level of the interdental septum beneath the tumor with a relatively intact periodontal cortical bone, exhibiting a teardrop-shaped bone defect. The lesion was completely resected with the periosteum.Gingival neurofibroma in NF-1 may be associated with osseous and dental abnormalities and can be mistaken for periodontitis. Raising awareness of this clinical entity can lead to proper management of the esthetic and functional problems in the oral and maxillofacial region.
牙龈增生虽然在临床环境中经常遇到，但很少与全身性疾病或综合征相关。在1型神经纤维瘤病（NF-1）的各种病理状况中，口面部区域的轻微表现偶尔会被忽视。在此，作者报告了1例NF-1患者发生牙龈神经纤维瘤的罕见病例，该患者自首次检查起17年的病程中伴有牙槽骨特征性骨缺损。一名患有NF-1的5岁男孩因左侧上颌后牙区牙龈增生前来就诊。切取活检确诊为神经纤维瘤。22岁时，该患者因同一区域出血和疼痛再次就诊，经牙周治疗无效。牙龈和结节肿胀，第二磨牙受肿瘤影响。影像学检查显示肿瘤下方的牙间间隔水平较低，牙周皮质骨相对完整，呈现泪滴状骨缺损。病变连同骨膜一起被完全切除。NF-1患者的牙龈神经纤维瘤可能与骨和牙齿异常有关，并且可能被误诊为牙周炎。提高对这种临床实体的认识可以导致对口颌面部区域美观和功能问题的妥善处理。
