Article
Radiation-induced Sarcomas Occurring in Desmoid-type Fibromatosis Are Not Always Derived From the Primary Tumor.
The American journal of surgical pathology - 1 Dec 2015
Verschoor Arie J, Cleton-Jansen Anne-Marie, Wijers-Koster Pauline, Coffin Cheryl M, Lazar Alexander J, Nout Remi A, Rubin Brian P, Gelderblom Hans, Bovée Judith V M G
Abstract excerpt
Desmoid-type fibromatosis is a rare, highly infiltrative, locally destructive neoplasm that does not metastasize, but recurs often after primary surgery. Activation of the Wnt/β-catenin pathway is the pathogenic mechanism, caused by an activating mutation in exon 3 of CTNNB1 (85% of the sporadic patients). Radiotherapy is a frequent treatment modality with a local control rate of approximately 80%. In very rare...
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