Rumi Shin1, Hwan-Seong Cho2, Duck-Woo Kim3,4, So Yeon Ahn5, Myong Hoon Ihn6, Hyo Jin Park7, Heung-Kwon Oh8, Sung-Bum Kang8. 1. Department of Surgery, Seoul Metropolitan Government-Seoul National University Boramae Medical Center, Seoul, South Korea. 2. Department of Orthopedic Surgery, Seoul National University Bundang Hospital, Seongnam, South Korea. 3. Department of Surgery, Seoul National University Bundang Hospital, 82, Gumi-ro 173beon-gil, Bundang-gu, Seongnam, 13620, South Korea. kdw@snubh.org. 4. Korean Hereditary Tumor Registry, Seoul National University College of Medicine, Seoul, South Korea. kdw@snubh.org. 5. Medical Research Collaborating Center, Seoul National University Bundang Hospital, Seongnam, South Korea. 6. Department of Surgery, Soonchunhyang University Gumi Hospital, Gumi, South Korea. 7. Department of Pathology, Seoul National University Bundang Hospital, Seongnam, South Korea. 8. Department of Surgery, Seoul National University Bundang Hospital, 82, Gumi-ro 173beon-gil, Bundang-gu, Seongnam, 13620, South Korea.
Abstract
PURPOSE: Desmoid tumor (DT) is a rare myofibroblastic proliferative neoplasm, but can occur in up to 26% of patients with familial adenomatous polyposis (FAP). Therefore, DT may be an extraintestinal manifestation of FAP that precedes the development of advanced malignancies. The aim of this study was to identify the incidence of polyps in DT patients and investigate value of colonoscopy for diagnosis of FAP in DT patients without gastrointestinal symptoms. MATERIALS AND METHODS: The records of patients diagnosed with DT were retrospectively reviewed using the clinical data warehouse (CDW) system. Clinical data, including location of tumor, type(s) of treatment, and colonoscopic findings, were collected. RESULTS: Sixty-five patients were diagnosed with DT during the study period; 10 patients received colonoscopies before diagnosis of DT, and 20 patients received colonoscopies after diagnosis of DT. The mean age at diagnosis of DT was 41.9 ± 16.7 years old (range 17-74). Most tumors were extra-abdominal (n = 24, 80.0%), and fewer were intra-abdominal (n = 4, 13.3%) or in an abdominal wall (n = 2, 6.7%). The colonoscopy results revealed one or two tubular adenomas in 11 patients (36.6%), although none of the patients had colorectal polyposis or colorectal cancer. CONCLUSION: None of the patients in our study population had colorectal polyposis. Routine colonoscopy of DT patients without gastrointestinal symptoms seems to have low diagnostic yield for detection of FAP.
PURPOSE:Desmoid tumor (DT) is a rare myofibroblastic proliferative neoplasm, but can occur in up to 26% of patients with familial adenomatous polyposis (FAP). Therefore, DT may be an extraintestinal manifestation of FAP that precedes the development of advanced malignancies. The aim of this study was to identify the incidence of polyps in DT patients and investigate value of colonoscopy for diagnosis of FAP in DT patients without gastrointestinal symptoms. MATERIALS AND METHODS: The records of patients diagnosed with DT were retrospectively reviewed using the clinical data warehouse (CDW) system. Clinical data, including location of tumor, type(s) of treatment, and colonoscopic findings, were collected. RESULTS: Sixty-five patients were diagnosed with DT during the study period; 10 patients received colonoscopies before diagnosis of DT, and 20 patients received colonoscopies after diagnosis of DT. The mean age at diagnosis of DT was 41.9 ± 16.7 years old (range 17-74). Most tumors were extra-abdominal (n = 24, 80.0%), and fewer were intra-abdominal (n = 4, 13.3%) or in an abdominal wall (n = 2, 6.7%). The colonoscopy results revealed one or two tubular adenomas in 11 patients (36.6%), although none of the patients had colorectal polyposis or colorectal cancer. CONCLUSION: None of the patients in our study population had colorectal polyposis. Routine colonoscopy of DT patients without gastrointestinal symptoms seems to have low diagnostic yield for detection of FAP.
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