Chemotherapy responsiveness in a patient with multiply relapsed ameloblastic fibro-odontosarcoma of the maxilla

Pediatr Blood Cancer. 2015 Nov;62(11):2029-32. doi: 10.1002/pbc.25627. Epub 2015 Jul 15.


Ameloblastic fibro-odontosarcoma (AFOS) is an extremely rare malignant odontogenic tumor. Complete surgical excision is the treatment of choice. Deaths due to disease recurrence and/or progression are documented. Here, we report the case of a 15-year-old female with multiple recurrent AFOS. She responded to chemotherapy with ifosfamide and doxorubicin consolidated by stereotactic reirradiation using cyberknife and remained in complete remission 14 months from the end of reirradiation therapy. Chemotherapy with ifosfamide and doxorubicin should be considered in advanced cases of AFOS.

Keywords: ameloblastic fibro-odontoma; ameloblastic fibro-odontosarcoma; chemotherapy response; radiotherapy; relapse; transformation.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Ameloblastoma / surgery*
  • Child
  • Female
  • Fibrosarcoma / surgery*
  • Humans
  • Maxillary Neoplasms / surgery*
  • Neoplasm Recurrence, Local / surgery*
  • Radiosurgery*
  • Remission Induction