title: [A Case of a Desmoid Tumor the Developed Around Ileostomy in a Patient with FAP].
abstract: A 21-year-old woman who underwent laparoscopic total colectomy for familial adenomatous polyposis (FAP) 1 year 3 months previously presented with a mass larger than 10 cm around the ileostomy. Multiple tumors in the mesentery around the ileostomy and anterior to the sacrum, accompanied by bilateral hydronephrosis, were detected by computed tomography. The patient was diagnosed with intraabdominal desmoid tumors, stage Ⅳ according to the Church's classification. The desmoid tumor (15×9 cm) around the ileostomy was completely resected surgically, whereas another desmoid tumor (5×4 cm) was incompletely resected. We found a desmoid tumor of more than 10 cm in size and many fibromatous plaques in the mesentery. We then performed 4 courses of systemic chemotherapy with dacarbazine and doxorubicin in for the residual desmoid tumors after surgery. There was no growth of the residual desmoid tumors for 12 months after chemotherapy. Genetic tests detected a pathogenic germline mutation of the APC gene in the high-risk region of the desmoid tumor. We also confirmed somatic mutations in the resected specimens.
authors.affiliation: Dept. of Digestive Tract and General Surgery, Saitama Medical Center, Saitama Medical University.
authors.forename: Noriyasu
authors.initials: N
authors.lastname: Chika
authors.forename: Kensuke
authors.initials: K
authors.lastname: Kumamoto
authors.forename: Okihide
authors.initials: O
authors.lastname: Suzuki
authors.forename: Azusa
authors.initials: A
authors.lastname: Yamamoto
authors.forename: Yusuke
authors.initials: Y
authors.lastname: Tajima
authors.forename: Yuichiro
authors.initials: Y
authors.lastname: Watanabe
authors.forename: Hisashi
authors.initials: H
authors.lastname: Onozawa
authors.forename: Takeaki
authors.initials: T
authors.lastname: Matsuzawa
authors.forename: Hidetaka
authors.initials: H
authors.lastname: Eguchi
authors.forename: Keiichiro
authors.initials: K
authors.lastname: Ishibashi
authors.forename: Erito
authors.initials: E
authors.lastname: Mochiki
authors.forename: Hideyuki
authors.initials: H
authors.lastname: Ishida
mesh_terms: D000008:Abdominal Neoplasms / Q000235:genetics / Q000473:pathology / Q000601:surgery*; D011125:Adenomatous Polyposis Coli / Q000601:surgery; D025601:Adenomatous Polyposis Coli Protein / Q000235:genetics; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D003606:Dacarbazine / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D005260:Female; D018222:Desmoid Tumors / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics / Q000601:surgery*; D006801:Humans; D007081:Ileostomy; D009154:Mutation; D055815:Young Adult
chemical_list: C555337:APC protein, human; D025601:Adenomatous Polyposis Coli Protein; D003606:Dacarbazine; D004317:Doxorubicin