Journal of Oral Sign
  • Year: 2013
  • Volume: 5
  • Issue: 2

An Unusual Bilateral Palatal Manifestation of Neurofibromatosis Type I -A Rare Case Report

  • Author:
  • Rajni Aggarwal1,, Manoj Garg2, Saurav Aggarwal3, Tushar Kakkar4
  • Total Page Count: 4
  • Page Number: 90 to 93

1Department of Periodontics, Surendera Dental College and Research Institute; Sriganganagar, Rajasthan

2Department of Surgery, B.J. Medical College and Hospital, Ahmedabad, Gujarat; India

3Department of Medicine, MM. College of Medical Sciences And Research Institute, Mullana; Haryana; India

4Department of Periodontics, Genesis Dental College And Research Institute; Ferozepur, Punjab; India

*Address for correspondence: Dr Rajni Aggarwal Senior Lecturer Department of Periodontics, Surendra Dental College And Research Institute; Sriganganagar, Rajasthan; India. E.mail: rajniperio@gmail.com, Mobile: 08233331282, 08233357916

Online published on 7 December, 2013.

Abstract

Neurofibroma is a benign peripheral nerve sheath tumour. It is one of the most frequent tumours of neural origin and its presence is one of the clinical criteria for the diagnosis of type 1 neurofibromatosis (NF-1) also known as Von Recklinghausen's disease. NF-1 is an autosomal dominantly inherited disease Caused by a germ-line–inactivating mutation in the NF1 gene on the long arm of chromosome 17. Oral manifestations have been reported in 4–7% cases of NF-1 but rarely observed on palate.

One of the most dreadful complications of NF-1 is development of sarcomatous change (neurofibrosarcoma), estimated to occur in about 5% of cases. Hence, it is important to recognize, identify the characteristics and clinically diagnose these lesions/manifestations so as to avoid the life threatening complications. This case report represents an unusual case of bilateral neurofibroma of the hard palate confirmed by histopathological examination after incisional biopsy.

Keywords

Neurofibromatosis Type 1, Von Recklinghausen disease, bilateral palatal neurofibroma