Oral manifestations of Type I Neurofibromatosis in a family

Abstract

Neurofibroma is a benign peripheral nerve sheath tumor. It is one of the most frequent tumors of neural origin and its presence is one of the clinical criteria for the diagnosis of neurofibromatosis type I (NF-I). Neurofibromatosis type I is an autosomal dominantly inherited disease due to an alteration in the long arm of chromosome 17. About 50% of NF-I patients have no family history of the disease. NF-I patients have skin lesions (café au lait spots and neurofibromas) as well as bone malformations and central nervous system tumors. Diagnosis is based on a series of clinical criteria. NF-I presents with certain definite oral manifestations which confers to the dentists a major responsibility for accurate diagnosis and report of the disease. The paper aims to highlight the role of an oral diagnostician in first recognition of NF-I in a family via manifestations in the oral cavity.

Common conditions associated with mandibular canal widening: A literature review

“…Controversy exists regarding the frequency of oral manifestations of neurofibroma. A frequency of 4%–7% has been reported by some, while values up to 72% have been reported by others 21. In most cases, multiple neurofibromas occur as part of neurofibromatosis.…”

“…Neurofibroma is the most common peripheral nerve tumor, although it rarely occurs in the oral cavity. It may manifest in 2 clinical forms: peripheral (type I) and central (type II) 2122. Controversy exists regarding the frequency of oral manifestations of neurofibroma.…”

“…However, solitary masses without visceral manifestations have also been reported 322. Neurofibromas of the oral cavity most often involve the tongue, followed by the lips, the palate, buccal mucosa, gingiva, floor of the mouth, and pharynx in descending order of frequency 21. Intraosseous lesions in the posterior mandible have also been reported to manifest as a well- or poorly-defined unilocular or multilocular radiolucency 22.…”

“…Other radiographic findings include lengthening, narrowing, or rarefaction of the coronoid and articular processes, deepening of the sigmoid notch, and enlargement of the mandibular canal (Fig. 4), mandibular foramen, or mental foramen 21. Visnapuu et al24 evaluated 102 neurofibromatosis patients and reported mandibular canal widening in 25%.…”

Von Recklinghausen's Disease Type I—A Case Report

“…The term neurofibromatosis is used for a group of genetic disorders that primarily affects the growth of neural tissues. 1 First described in 1882, by the German anatomo-pathologist, Von Recklinghausen, it is an autosomal dominant disorder that affects the bone, nervous system, soft-tissue, and the skin. 2 It is caused by a spectrum of mutations that affect the gene located on the 17q11.2 chromosome, known as the NFI gene.…”