Güneri Enis Alpin, Akoğlu Ertap, Sütay Semih, Ceryan Kerim, Sağol Ozgül, Pabuçcuoğlu Uğur
Department of Oto-Rhino-Laryngology, Head and Neck Surgery, Dokuz Eylül University Faculty of Medicine, Izmir, Turkey.
Turk J Pediatr. 2006 Apr-Jun;48(2):155-8.
A three-year-old girl with a lingual plexiform neurofibroma treated by total excision is presented. Despite their occurrence in the head and neck region, neural sheath tumors are rarely encountered in the oral cavity. It is reported that 4-7% of patients affected by neurofibromatosis display oral manifestations. Neurofibromatosis is characterized by café-au-lait spots and cutaneous neurofibromas. Plexiform neurofibroma is said to be indicative of von Recklinghausen's disease (VRD) even though it may be the only manifestation of the disease. Generally, surgical resection represents the treatment of choice and the diagnosis can only be confirmed after histological examination. Affected patients need regular follow-up to detect malignant degeneration, an early recurrence or appearance of other manifestations of VRD.
本文介绍了一名通过全切术治疗的患有舌部丛状神经纤维瘤的三岁女孩。尽管神经鞘瘤发生于头颈部区域,但在口腔中却很少见。据报道,4%-7%的神经纤维瘤病患者有口腔表现。神经纤维瘤病的特征为咖啡斑和皮肤神经纤维瘤。丛状神经纤维瘤据说即使可能是该疾病的唯一表现,也提示冯雷克林霍增氏病(VRD)。一般来说,手术切除是首选治疗方法,且只有在组织学检查后才能确诊。患病患者需要定期随访,以检测恶性变、早期复发或VRD其他表现的出现。