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Osteosarcoma
1%
47/3379
Leiomyosarcoma
26%
867/3379
Extraabdominal fibromatoses (desmoid tumor)
68%
2313/3379
Angiosarcoma
3%
115/3379
Chondroblastoma
0%
15/3379
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The clinical presentation and imaging studies are consistent with extraabdominal fibromatoses, also known as a desmoid tumor. Extraabdominal fibromatoses (desmoid tumor) is an infiltrative tumor composed of fibroblasts and myofibroblasts that, despite high incidence of local recurrence, does not metastasize. It occurs in individuals 15-40 years of age and is 10,000 times more likely to develop in patients with familial adenomatous polyposis (FAP) syndrome. Treatment is typically wide resection much like soft tissue sarcoma. Deyrup et al reviewed 40 cases of extraabdominal fibromatosis and found that 100% of the patients were positive for estrogen receptor-beta expression and 100% of the cases were negative for estrogen receptor-alpha expression. Illustration A details the histopathology of the desmoid tumor in this case.
2.9
(23)
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