Plexiform neurofibroma with neurofibromatosis type I/ von Recklinghausen's disease: A rare case report.

ABSTRACT:

Introduction

Plexiform neurofibroma with neurofibromatosis type 1 (NF1) or Von Recklinghausen's disease is a rare entity and occurs in approximately 5-15% patients. These are slow growing, painless and locally infiltrating tumors. The pattern of inheritance is autosomal dominant and its penetrance is almost complete by 5 years of age.

Presentation of case

We hereby report a case of 13 years old boy visited presenting with swelling of right eyelid and forehead. After surgical removal, the tissue was sent for histopathological evaluation. Microscopy revealed an unencapsulated tumor mass comprising of well organized mixture of multiple nerve bundles with interlacing neural tissue in background of spindle shaped cells along with myxoid areas and numerous blood vessels.

Discus

SUBMITTER: Poswal P 

PROVIDER: S-EPMC7473834 | biostudies-literature | 2020 Sep

REPOSITORIES: biostudies-literature