Bhoyar Nidhi, Gupta Sunita, Ghosh Sujoy
Department of Oral Medicine and Radiology, Maulana Azad Institute of Dental Sciences, New Delhi, India.
Contemp Clin Dent. 2016 Oct-Dec;7(4):558-561. doi: 10.4103/0976-237X.194120.
Tuberous sclerosis complex (TSC) is a rare multisystem genetic disease, with an estimated incidence of 1 in 6000-1 in 10,000. TSC is an autosomal dominant syndrome involving heart, kidneys, lungs, and skin. The classic triad of TSC is seizures, mental retardation, and angiofibromas; this triad occurs in only 29% of patients. The clinical diagnostic guidelines on TSC are prepared based on clinical features, radiographic findings. The most common oral manifestations of TSC are fibroma, gingival hyperplasia, and enamel hypoplasia. Odontogenic myxofibroma represents a rare slow-growing benign neoplasm found rarely in children below 10 years or adults over 50 years of age. The prevalence of myxoma is between 0.04% and 3.7%. Here, we are reporting a rare case of myxofibroma of gingiva in an 8-year-old female TSC patient.
结节性硬化症（TSC）是一种罕见的多系统遗传性疾病，估计发病率为1/6000至1/10000。TSC是一种常染色体显性综合征，累及心脏、肾脏、肺和皮肤。TSC的典型三联征是癫痫发作、智力迟钝和血管纤维瘤；仅29%的患者出现此三联征。TSC的临床诊断指南是根据临床特征、影像学检查结果制定的。TSC最常见的口腔表现是纤维瘤、牙龈增生和釉质发育不全。牙源性黏液纤维瘤是一种罕见的生长缓慢的良性肿瘤，很少见于10岁以下儿童或50岁以上成人。黏液瘤的患病率在0.04%至3.7%之间。在此，我们报告一例8岁女性TSC患者发生牙龈黏液纤维瘤的罕见病例。
