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[Isolated sub-orbital plexiform neurofibroma].

L Chikhani1, M Divaris, M Rosenstiel

  • 1Institut de Stomatologie, Chirurgie plastique et chirurgie maxillo-faciale, Paris.

Revue De Stomatologie Et De Chirurgie Maxillo-Faciale
|January 1, 1992
PubMed
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A rare suborbital plexiform neurofibroma in a 73-year-old patient indicated a minor form of von Recklinghausen's disease. This finding highlights the varied cephalic manifestations of this genetic disorder.

Area of Science:

  • Neurology
  • Oncology
  • Dermatology

Background:

  • Von Recklinghausen's disease, also known as neurofibromatosis type 1 (NF1), is a genetic disorder.
  • It causes tumors to grow on nerves and can affect skin, bone, and soft tissues.
  • Cephalic manifestations are common but varied in NF1.

Observation:

  • Histological examination of a 73-year-old patient revealed an unexpected isolated suborbital plexiform neurofibroma.
  • This specific tumor type is a key indicator for neurofibromatosis.

Findings:

  • The presence of an isolated suborbital plexiform neurofibroma led to the diagnosis of a minor form of von Recklinghausen's disease.
  • This case underscores that even isolated findings can signify underlying genetic conditions.

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Implications:

  • Early identification of neurofibromas, even in unusual locations, is crucial for diagnosing NF1.
  • Understanding the spectrum of cephalic manifestations aids in comprehensive patient management.
  • This case contributes to the literature on the diverse clinical presentations of neurofibromatosis type 1.