title: Unusual limbal lesion in neurofibromatosis type 1.
abstract: OBJECTIVE
  To describe an unusual limbal lesion clinically resembling pterygium in a young patient with neurofibromatosis type 1.
  
  
  METHODS
  Clinical case report with a review of the literature.
  
  
  RESULTS
  The lesion was excised, and histopathology was performed. Histopathology disclosed conjunctival mucosa with expansion of the substantia propria by dense and loose collagenous tissue, small nerves, and thin-walled blood vessels. No skin or skin appendages were seen, although a small amount of mature adipose was present. Focally, spindle cells with wavy nuclei were seen scattered within the collagenous areas and were positive on S100 immunohistochemistry. Mast cells were readily identifiable. There was no evidence of actinic elastosis, dysplasia, or malignancy. These histopathological features most resemble heterotopia with some features of neurofibroma.
  
  
  CONCLUSIONS
  We report an unusual limbal lesion in a patient with neurofibromatosis type 1. Histopathological features resemble a heterotopia with some neurofibroma features. We suggest that all lesions excised from patients with neurofibromatosis, no matter how characteristic in appearance, be sent for histopathological confirmation of diagnosis.
authors.affiliation: *Save Sight Institute, Sydney University, Australia; and †University of New South Wales, Australia.
authors.forename: Chameen
authors.initials: C
authors.lastname: Samarawickrama
authors.forename: Svetlana
authors.initials: S
authors.lastname: Cherepanoff
authors.forename: Nick
authors.initials: N
authors.lastname: Di Girolamo
authors.forename: Ross
authors.initials: R
authors.lastname: Benger
authors.forename: Stephanie
authors.initials: S
authors.lastname: Watson
mesh_terms: D000293:Adolescent; D003316:Corneal Diseases / Q000473:pathology*; D005134:Eye Neoplasms / Q000473:pathology*; D006801:Humans; D016850:Limbus Corneae / Q000473:pathology*; D008297:Male; D009455:Neurofibroma / Q000473:pathology*; D009456:Neurofibromatosis 1 / Q000473:pathology*