Article
Rectal leiomyosarcoma as the initial phenotypic manifestation of Li-Fraumeni-like syndrome: a case report and review of the literature.
Journal of medical case reports - 19 Dec 2022
Severino Natalia Parisi, Waisberg Jaques, Fragoso Maria Candida Barisson Villares, de Lima Luiz Guilherme Cernaglia Aureliano, Balsamo Flavia, Henriques Alexandre Cruz, Bianco Bianca, de Sousa Gehrke Flávia
Abstract excerpt
BACKGROUND: Leiomyosarcoma is a rare malignant tumor of smooth muscle origin and represents 10-20% of all soft tissue sarcomas. Primary colon and rectal sarcomas constitute < 0.1% of all large bowel malignancies. In Li-Fraumeni syndrome, sarcomas are the second most frequent cancer (25%). Li-Fraumeni syndrome is a genetic disease with a familial predisposition to multiple malignant neoplasms. This syndrome has an...
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