The Features of Colorectal Tumors in a Patient with Li-Fraumeni Syndrome

A young woman with Li-Fraumeni syndrome (LFS) was referred to our hospital. On examination, multiple flat neoplasms were detected in addition to semi-pedunculated polyps. Restorative proctocolectomy was performed; one submucosal invasive cancer, two mucosal cancers, and several adenomas with high-gr...

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Detalles Bibliográficos
Autores principales: Yoshida, Tsukasa, Tajika, Masahiro, Tanaka, Tsutomu, Ishihara, Makoto, Hirayama, Yutaka, Mizuno, Nobumasa, Hara, Kazuo, Hijioka, Susumu, Imaoka, Hiroshi, Hieda, Nobuhiro, Okuno, Nozomi, Kinoshita, Takashi, Bhatia, Vikram, Shimizu, Yasuhiro, Yatabe, Yasushi, Yamao, Kenji, Niwa, Yasumasa
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Japanese Society of Internal Medicine 2017
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5348453/
https://www.ncbi.nlm.nih.gov/pubmed/28154273
Descripción
Sumario:A young woman with Li-Fraumeni syndrome (LFS) was referred to our hospital. On examination, multiple flat neoplasms were detected in addition to semi-pedunculated polyps. Restorative proctocolectomy was performed; one submucosal invasive cancer, two mucosal cancers, and several adenomas with high-grade dysplasia were detected. On immunohistochemical staining with p53, every part of all neoplasms, even the small adenomas, showed strong positive staining. Multiple flat neoplasms may be characteristic of patients with LFS and may have a much higher risk of rapid progression to invasive carcinomas than sporadic neoplasms. Thus, careful and frequent colonoscopy surveillance may be needed for patients with LFS.

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