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Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2007 Apr;103(4):e54-9. Epub 2006 Sep 26.

Malignant fibrous histiocytoma (undifferentiated high-grade pleomorphic sarcoma) occurring in tuberous sclerosis: a case report.

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1
Pathologist, Lauro de Souza Lima Institute, Bauru, São Paulo, Brazil.

Abstract

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.

PMID:
17395060
DOI:
10.1016/j.tripleo.2006.05.014
[Indexed for MEDLINE]
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