Birt-Hogg-Dubé syndrome: two patients with neural tissue tumors

J Am Acad Dermatol. 2003 Oct;49(4):717-9. doi: 10.1067/s0190-9622(03)01583-4.


We present 2 unrelated patients found to have tumors of neural tissue origin, a neurothekeoma and a meningioma, who were additionally diagnosed with the syndrome of Birt-Hogg-Dubé (BHDS). We are unaware of previous BHDS patients with neural tissue tumors. In light of recent linkage analysis studies delineating the genetic susceptibility locus for BHDS, we speculate about a possible association between BHDS and neural tissue tumors.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Hamartoma Syndrome, Multiple / genetics*
  • Humans
  • Meningioma / genetics*
  • Middle Aged
  • Neoplasms, Multiple Primary / genetics*
  • Neurothekeoma / genetics*
  • Nose Neoplasms / genetics*
  • Skin Neoplasms / genetics*