Microdont Developing Outside the Alveolar Process and Within Oral Diffuse and Plexiform Neurofibroma in Neurofibromatosis Type 1

Abstract

Background/Aim: Numerical aberrations of permanent dentition and dystopic tooth eruption are part of the phenotype of the tumor predisposition syndrome neurofibromatosis type 1 (NF1). In these cases, surplus tooth germs usually develop in the alveolar processes of the jaw. This report attests to the dystopic development of a dysplastic supernumerary tooth in NF1 arising outside the jaw. Case Report: The 8-year-old male patient developed a microdont outside the bone and above the occlusal plane of the retained maxillary right second molar. The supernumerary tooth was completely embedded in oral soft tissue. Hyperplastic oral soft tissue in the molar region and microdont were excised. Specimen of the mucosa surrounding the teeth was interspersed with diffuse and plexiform neurofibroma. The retained upper right first molar emerged spontaneously within a few months after surgery. The upper right second molar did not change position. Conclusion: Odontogenesis can take place within tumorous oral mucosa in NF1. Surgical removal of the tumorous mucous membrane facilitates tooth eruption in some cases.Numerical aberrations of the permanent dentition have been reported in patients with neurofibromatosis type 1 (NF1) (1). NF1-associated supernumerary teeth were found more frequently in the molar region than in other tooth types (2,3). A topographic relationship between dental numerical aberrations and nerve sheath tumors typically arising in NF1 has been noticed in some cases (3). However, supernumerary teeth are regarded a constitutive finding in NF1 (3). This case report describes the diagnosis and treatment of a patient with NF1 who developed a supernumerary tooth of small size.

Characterization of Dental Pulp Stem Cell Populations in the Teeth of Patients With Neurofibromatosis Type 1 - Therapeutic Potential for Bone Tissue Engineering

“…Obtaining and further processing of DPSCs was not influenced by topographical peculiarities. Analogous to the apparently lack of influence of PNF in NF1 on the shape (27) and number of teeth (28,29), and the capacity of oral diffuse-plexiform neurofibroma to differentiate extraosseous displaced odontogenic tissue to a tooth crown (30), DPSCs derived from teeth embedded in an oral PNF can be differentiated in the same way as teeth without any apparent contact to a PNST. In both situations, NF1 derived DPSCs differentiate identical to controls.…”

“…The enlarged mandibular foramen is a radiologically conspicuous sign of a dorsal differentiation disorder through an adjacent PNF (36), as is the relatively frequently reported enlargement of the mandibular canal (37). However, the development of teeth in the orofacial PNF region is often disturbed in that the mesial migration of certain permanent teeth is incomplete or completely absent and the roots of the teeth are deformed due to the lack of bony space, but the visible structure of the tooth is not impaired (27).…”

Imaging characteristics of orbital peripheral nerve sheath tumors: Analysis of 34 cases

“…Based on five large studies of biopsy-proven orbital tumors, Sweeney et al [ 10 ] reported the occurrence probability of PNSTs in different orbital tumors: schwannoma 0.7%-2.3%, neurofibroma 0.4%-3.0%, malignant PNST 0%-0.2%. Unlike malignant PNSTs, which have a peak incidence in the seventh decade, benign orbital PNSTs are considered tumors of adulthood, with the exception of plexiform neurofibromas, of which approximately 50% are diagnosed in early childhood[ 13 , 14 ]. In our study, the mean age of patients with schwannoma and neurofibroma was 47.33 ± 3.602 years and 42.17 ± 4.977 years, respectively.…”

Diffuse Neurofibroma in a Micronesian Male

“…Despite the hyperpigmentation seen clinically, the negative MART-1 stain excludes classifying this diffuse neurofibroma as melanotic. This distinction is crucial as there have been reported cases of melanotic plexiform neurofibroma [5]. Imaging identified a rim-enhancing fluid collection, raising concerns regarding a necrotic tumor or an infected fluid collection.…”