Neurofibromatosisban szenvedő anya hétéves leánya alsó állcsontjának középvonalában kifejlődő, klinikailag gingivalis hyperplasia képében jelentkező neurofibroma

Abstract

A seven-year-old girl was referred to the Department of Periodontology of the Semmelweis University with a symmetric bilateral, painless, non-inflammatory diffuse enlargement on the lingual aspects of her lower jaw. The family history revealed that her mother and elder sister had Recklinghausen's disease with typical characteristic dermatological signs and they are registered by the National NF Registry. Extraoral examination revealed an evident protrusion of the lips. Intraorally, the buccal gingiva around the upper and lower teeth appeared normal. The little patient had neither dermal nor oral mucous membrane signs characteristic of NF1. With the consent of her parents, the lingual firm mass of gingival enlargement was excised under local anesthesia and the removed tissue was histologically analyzed. The histology approved the clinical diagnosis as solitary neurofibroma. The postoperative healing was uneventful. Because of her very young age and the subtotal excision of the tumor, the patient has been regularly monitored. After one and a half years, recurrence or other sign of tumor regrowth have not been observed. The panoramic radiograph showed normal bone morphology and an age-related dental status with mixed dentition and undisturbed tooth eruption. The present case with a midline bilateral diffuse gingival overgrowth in the mouth of a 7-year-old girl without any characteristic dermatological signs in a family with genetically proven Recklinghausen's disease is very rare and unique in the literature. Orv Hetil. 2020; 161(22): 924-930.