Li-Fraumeni syndrome, characterised by presence of different multiple primary tumors Degisik orijinli multipl primer tumorle karakterize 'Li-Fraumeni' sendromu

KIRIŞTIOĞLU İ. , KILIÇ N. , Turkel T., Dogruyol H.

Pediatrik Cerrahi Dergisi, vol.12, pp.54-56, 1998 (Refereed Journals of Other Institutions) identifier

  • Publication Type: Article / Article
  • Volume: 12
  • Publication Date: 1998
  • Title of Journal : Pediatrik Cerrahi Dergisi
  • Page Numbers: pp.54-56


Li-Fraumeni familial cancer syndrome is a disorder that predisposes individuals to multiple forms of cancer including breast cancer, brain tumor and adrenocortical carcinoma. In this study we present a patient with Li- Fraumeni syndrome who has an extremity rhabdomyosarcoma (RMS) and phecromacytoma as a second primary. The sister of the patient showed bilateral adrenocortical tumors. In this report, the importance of the presence of a second tumor and family screening in the follow-up of childhood rhabdomyosarcoma is stressed.