title: Pancreatic desmoid-type fibromatosis with beta-catenin gene mutation-Report of a case and review of the literature.
abstract: We experienced a rare case of pancreatic desmoid-type fibromatosis (DTF) in a 75-year-old Japanese woman. She was asymptomatic but routine examination including ultrasonography revealed a mass in the abdomen. For precise examination, she was referred to the regional hospital. Computed tomography showed that the mass was protruding anteriorly from the left-sided pancreas. Because of the enlargement of the mass lesion, distal pancreatectomy with splenectomy was performed after about 3 months. Macroscopically, the mass was encapsulated and approximately 8cm in diameter. Histological examination revealed that spindle or blunt stellate cells were proliferating in parallel or storiform fashion with myxoid and fibrous background. The tumor cells did not show prominent atypia and mitoses were rarely seen, suggesting that the tumor was low grade or borderline. Immunohistochemistry showed obvious nuclear staining of beta-catenin. Furthermore, analysis of beta-catenin gene revealed that the tumor had a typical missense mutation of threonine to alanine at colon 41 (T41A) in exon 3. These findings confirmed the pathological diagnosis of DTF of the pancreas. To the best of our knowledge, 18 cases of pancreatic DTF have been reported in the English literature and beta-catenin gene mutation had been examined in only one case among them. Thus, our case is the 19th pancreatic DTF and the second case with confirmed beta-catenin gene mutation.
authors.affiliation: Department of Surgical Pathology, Hyogo College of Medicine, 1-1 Mukogawa-cho, Nishinomiya 663-8501, Hyogo, Japan. Electronic address: tsuka-y@hyo-med.ac.jp.
authors.forename: Yoshitane
authors.initials: Y
authors.lastname: Tsukamoto
authors.affiliation: Department of Pathology, Rinku General Medical Center, 2-23 Rinku-Ourai Kita, Izumisano-City 598-8577, Osaka, Japan.
authors.forename: Masami
authors.initials: M
authors.lastname: Imakita
authors.affiliation: Department of Surgery, Rinku General Medical Center, 2-23 Rinku-Ourai Kita, Izumisano-City 598-8577, Osaka, Japan.
authors.forename: Akiko
authors.initials: A
authors.lastname: Nishitani
authors.affiliation: Department of Surgery, Rinku General Medical Center, 2-23 Rinku-Ourai Kita, Izumisano-City 598-8577, Osaka, Japan.
authors.forename: Toshikazu
authors.initials: T
authors.lastname: Ito
authors.affiliation: Department of Surgery, Rinku General Medical Center, 2-23 Rinku-Ourai Kita, Izumisano-City 598-8577, Osaka, Japan.
authors.forename: Masaaki
authors.initials: M
authors.lastname: Izukura
authors.affiliation: Department of Surgical Pathology, Hyogo College of Medicine, 1-1 Mukogawa-cho, Nishinomiya 663-8501, Hyogo, Japan.
authors.forename: Seiichi
authors.initials: S
authors.lastname: Hirota
mesh_terms: D005260:Female; D018222:Desmoid Tumors / Q000000981:diagnostic imaging / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D006801:Humans; D009154:Mutation; D010180:Pancreatectomy; D010190:Pancreatic Neoplasms / Q000000981:diagnostic imaging / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D013156:Splenectomy; D014463:Ultrasonography; D051176:beta Catenin / Q000235:genetics*
chemical_list: D051176:beta Catenin
keywords: Beta-catenin; Desmoid-type fibromatosis; Mutation; Pancreas