家族性大腸腺腫症に合併するデスモイド腫瘍の診断・治療と重症度分類の提案

石田 秀行; 近谷 賢一; 母里 淑子; 百瀬 修二; 長田 久人; 山野 智基; 冨田 尚裕; 秋山 泰樹; 平田 敬治; 六車 直樹; 高山 哲治; 西田 佳弘; 石川 秀樹

doi:10.18976/jsht.20.2_45

Review

Diagnosis, treatment, and proposal of the new classification system for the severity of desmoid tumors associated with familial adenomatous polyposis

Hideyuki Ishida , Kenichi Chikatani, Yoshiko Mori, Syuji Momose, Hisato Osada, Tomoki Yamano, Naohiro Tomita, Yasuki Akiyama, Keiji Hirata, Naoki Nuguruma, Tetsuji Takayama, Yoshihiro Nishida, Hideki Ishikawa

Author information

Hideyuki Ishida [Japan] Corresponding author
Department of Digestive Tract and General Surgery, Saitama Medical Center, Saitama Medical University, Kawagoe, Japan. [Japan]
Department of Clinical Genomics, Saitama Medical Center, Saitama Medical University, Kawagoe, Japan. [Japan]
Kenichi Chikatani [Japan]
Department of Digestive Tract and General Surgery, Saitama Medical Center, Saitama Medical University, Kawagoe, Japan. [Japan]
Yoshiko Mori [Japan]
Department of Digestive Tract and General Surgery, Saitama Medical Center, Saitama Medical University, Kawagoe, Japan. [Japan]
Department of Clinical Genomics, Saitama Medical Center, Saitama Medical University, Kawagoe, Japan. [Japan]
Syuji Momose [Japan]
Department of Pathology, Saitama Medical Center, Saitama Medical University, Kawagoe, Japan. [Japan]
Hisato Osada [Japan]
Department of Radiology, Saitama Medical Center, Saitama Medical University, Kawagoe, Japan. [Japan]
Tomoki Yamano [Japan]
Division of Lower GI Surgery, Department of Surgery, Hyogo College of Medicine, Nishinomiya, Japan. [Japan]
Naohiro Tomita [Japan]
Division of Lower GI Surgery, Department of Surgery, Hyogo College of Medicine, Nishinomiya, Japan. [Japan]
Yasuki Akiyama [Japan]
Department of Surgery 1, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan. [Japan]
Keiji Hirata [Japan]
Department of Surgery 1, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan. [Japan]
Naoki Nuguruma [Japan]
Department of Gastroenterology and Oncology, Tokushima University Graduate School of Biomedical Sciences, Tokushima, Japan. [Japan]
Tetsuji Takayama [Japan]
Department of Gastroenterology and Oncology, Tokushima University Graduate School of Biomedical Sciences, Tokushima, Japan. [Japan]
Yoshihiro Nishida [Japan]
Department of Rehabilitation, Nagoya University Hospital, Nagoya, Japan. [Japan]
Hideki Ishikawa
Department of Molecular-Targeting Prevention, Kyoto Prefectural University of Medicine, Kyoto, Japan. [Japan]

Keywords: Familial adenomatous polyposis, Desmoid tumor

JOURNAL OPEN ACCESS

2020 Volume 20 Issue 2 Pages 45-58

DOI https://doi.org/10.18976/jsht.20.2_45

Details

Abstract

Familial adenomatous polyposis (FAP) is an autosomal dominant inherited disorder characterized by multiple colorectal adenomas. Desmoid tumors (DT) are a well-known extracolonic manifestation of FAP that frequently develop in the mesentery, retroperitoneum, and/or abdominal wall in patients with FAP. DT negatively impact the quality of life and affect survival. Due to their unclear natural course and insufficient data, there is no established evidencebased medical treatment for DT. In this article, we describe the diagnosis and treatment for DT in patients with FAP, and propose a classification system for the severity of FAP-related DT, with the aim of establishing a standard treatment for this condition.

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