Malignant fibrous histiocytoma (undifferentiated high-grade pleomorphic sarcoma) occurring in tuberous sclerosis: a case report.
Tuberous sclerosis is an autosomal dominant genetic disorder that is characterized by the early development of hamartomas, malformations, and congenital tumors of the nervous system, skin, kidneys, lungs, and heart. Variable clinical expressivity has been reported and more than one gene can be responsible for the disorder. Benign neoplasias accompanying the syndrome are common but malignant neoplasias are rare, being generally mesenchymal and possibly affecting the jaws. We report here a clinical case of a malignant fibrous histiocytoma (undifferentiated high-grade pleomorphic sarcoma) of the mandible in a patient with tuberous sclerosis.
Fleury
Raul Negrão
RN
Pathologist, Lauro de Souza Lima Institute, Bauru, São Paulo, Brazil.
Damante
José Humberto
JH
Soares
Cleverson Teixeira
CT
Sant'Ana
Eduardo
E
Mello
Edgard José Franco
EJ
Moreira
Carla Ruffeil
CR
D000293:Adolescent; D001706:Biopsy; D003937:Diagnosis, Differential; D005887:Gingival Neoplasms / Q000150:complications / Q000473:pathology* / Q000601:surgery; D051677:Histiocytoma, Malignant Fibrous / Q000150:complications / Q000473:pathology* / Q000601:surgery; D006801:Humans; D008297:Male; D008339:Mandibular Neoplasms / Q000473:pathology* / Q000601:surgery; D009364:Neoplasm Recurrence, Local / Q000473:pathology* / Q000601:surgery; D014402:Tuberous Sclerosis / Q000150:complications*