Elguezabal Rodriguez Analía, Gené Hijós Miriam, Caro Tarragó Aleidis, Martínez González Salomé
Servicio de Patología, Hospital Universitario Joan XXIII, Tarragona, España.
Gastroenterol Hepatol. 2011 Jun-Jul;34(6):406-10. doi: 10.1016/j.gastrohep.2011.03.004. Epub 2011 May 14.
Fibromatosis consists of a benign fibroblastic proliferation with local infiltrative growth. Two types are recognized: a superficial and a deep form, also known as desmoid tumor. The latter may occur sporadically or in association with familial adenomatous polyposis and Gardner's syndrome. Pancreatic presentation is exceptional and only eight cases have been described in the literature.
We report the case of a 29-year-old woman with a history of familial colonic polyposis and two pancreatic lesions. In the surgical specimen, two poorly defined pancreatic lesions were observed with infiltration of neighboring organs. Histologically, the lesions corresponded to mesenchymal proliferation with a fusocellular pattern without cytological atypica, which were diagnosed as desmoid tumors.
The etiology of fibromatosis is unknown. In patients with familial colonic polyposis, the most common localization of desmoid tumor is intra-abdominal. Pancreatic presentation is unusual, requiring differential diagnosis with other pancreatic neoplasms.
纤维瘤病由具有局部浸润性生长的良性成纤维细胞增殖构成。可分为两种类型：浅表型和深部型，后者也称为硬纤维瘤。硬纤维瘤可散发性发生，或与家族性腺瘤性息肉病及加德纳综合征相关。胰腺纤维瘤病的表现极为罕见，文献中仅报道过8例。
我们报告一例29岁女性，有家族性结肠息肉病史，胰腺有两处病变。手术标本中观察到两处边界不清的胰腺病变，伴有邻近器官浸润。组织学检查显示，病变为间叶组织增殖，呈梭形细胞形态，无细胞学异型性，诊断为硬纤维瘤。
纤维瘤病的病因尚不清楚。在家族性结肠息肉病患者中，硬纤维瘤最常见的部位是腹腔内。胰腺纤维瘤病的表现不常见，需要与其他胰腺肿瘤进行鉴别诊断。
