Anaplastic ameloblastic fibrosarcoma arising from recurrent ameloblastic fibroma: restricted molecular abnormalities of certain genes to the malignant transformation.
A rare case of anaplastic ameloblastic fibrosarcoma (AS) arising in an ameloblastic fibroma (AF) of the maxilla of a 48-year-old patient 10 years after the primary excision is presented. The recurrent tumor retained focal areas of AF but manifested heterogeneous malignant features ranging from low-grade spindle to highly pleomorphic sarcomas. Biomarker analysis showed alterations of the p53 and c-KIT genes restricted to the sarcomatous component. The biological implications of these findings in the future management of these tumors are discussed.
Williams
Michelle D
MD
Department of Pathology, University of Texas M. D. Anderson Cancer Center, Houston, TX 77030, USA. anaggar@mdanderson.org
Hanna
Ehab Y
EY
El-Naggar
Adel K
AK
D005354:Fibrosarcoma / Q000235:genetics / Q000473:pathology*; D016158:Genes, p53; D006801:Humans; D008297:Male; D008441:Maxillary Neoplasms / Q000473:pathology* / Q000601:surgery; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000235:genetics / Q000473:pathology*; D009378:Neoplasms, Multiple Primary / Q000235:genetics / Q000473:pathology*; D009810:Odontoma / Q000473:pathology* / Q000601:surgery; D019009:Proto-Oncogene Proteins c-kit / Q000235:genetics
D019009:Proto-Oncogene Proteins c-kit