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Familial multiple neurilemoma. A case report.

F Yamazaki1, S Takahashi, M Andou

  • 1Department of Orthopedic Surgery, Kantou Rousai Hospital, Kawasaki, Japan.

Archives of Orthopaedic and Traumatic Surgery. Archiv Fur Orthopadische Und Unfall-Chirurgie
|January 1, 1988
PubMed
Summary
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This study reports a rare case of familial multiple neurilemoma in a 59-year-old man. The findings suggest a possible forme fruste or related condition to von Recklinghausen's disease.

Area of Science:

  • Neurology
  • Genetics
  • Oncology

Background:

  • Neurilemomas are typically benign nerve sheath tumors.
  • Familial occurrence of neurilemomas is rare, often associated with genetic predispositions.
  • Von Recklinghausen's disease (neurofibromatosis type 1) is a common genetic disorder associated with tumors.

Observation:

  • A 59-year-old man presented with five neurilemmomas.
  • A significant family history of soft-tissue tumors was noted, with 15 of 22 relatives affected.
  • Microscopic diagnosis confirmed ten neurilemmomas in five family members.

Findings:

  • The affected family members showed no typical symptoms of von Recklinghausen's disease.
  • The case presented appears to be a forme fruste or a related variant of neurofibromatosis.

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  • Genetic analysis may be warranted to identify specific mutations.

    • Implications:

    • This case expands the understanding of familial neurilemmoma and its genetic basis.
    • It highlights the importance of family history in diagnosing rare tumor syndromes.
    • Further research could clarify the relationship between familial neurilemmoma and neurofibromatosis spectrum disorders.