Intraorbital plexiform neurofibroma in an NF-1-negative patient

Siegfried Jank*, E. J. Raubenheimer, M. R. Bouckaert, P. Obrist, G. Bodner, A. Rudisch, I. Baldissera, K. Wimmer, H. Strobl

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

6 Citations (Scopus)


A 52-year-old patient presented with an orbital swelling and exophthalmos that enlarged over a period of about 40 years. The clinical examination showed massive exophthalmos and ptosis of the right eye without diplopia. The radiological investigation (MRI, CT and ultrasound) showed an unclear intraorbital mass with erosion of the orbital floor, infraorbital rim and orbital roof. The lesion was diagnosed histologically as a plexiform neurofibroma. The patient did not present any features of neurofibromatosis type 1 (NF-1) and molecular genetic analysis was unable to uncover a pathogenic sequence alteration in the NF-1 gene. Owing to the absence of clinical and ophthalmologic symptoms and the improbability of complete removal, the patient refused surgical intervention.

Original languageEnglish
Pages (from-to)240-244
Number of pages5
JournalDentomaxillofacial Radiology
Issue number4
Publication statusPublished - May 2007


  • Exophthalmos
  • Magnetic resonance imaging (MRI)
  • Neurofibromatosis type 1 (NF-1)
  • Orbit
  • Plexiform neurofibroma
  • Von Recklinghausen's disease


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