Desmoplastic fibroma-like tumor of maxillofacial region associated with tuberous sclerosis.
Desmoplastic fibroma is a rare primary tumor of bone that histologically and biologically mimics the extra-abdominal desmoid tumor of soft tissue. It usually presents in patients during the first three decades of life and often involves the mandible or long bones of the skeleton. Its clinical behavior is characterized by a locally aggressive, infiltrating, and destructing course, often with invasion of surrounding tissues but without metastasis. We present herein the clinicopathological features of a desmoplastic fibroma-like tumor involving the left maxillofacial region in a 14-year-old Hispanic boy with tuberous sclerosis.
Vargas-Gonzalez
Roberto
R
Escuela de Medicina Universidad Popular Autónoma del Estado de Puebla and Department of Pathology, Hospital Para el Niño Poblano, Puebla, México. soncoy@msn.com.
San Martin-Brieke
Walter
W
Gil-Orduña
Claudia
C
Lara-Hernandez
Fabiola
F
D000293:Adolescent; D001859:Bone Neoplasms / Q000150:complications / Q000473:pathology* / Q000601:surgery; D003937:Diagnosis, Differential; D018220:Fibroma, Desmoplastic / Q000150:complications / Q000473:pathology* / Q000601:surgery; D006801:Humans; D007573:Jaw Neoplasms / Q000150:complications / Q000473:pathology* / Q000601:surgery; D008297:Male; D014402:Tuberous Sclerosis / Q000150:complications*