Tumor desmoide intraabdominal simulando una tumoración pancreática en una paciente con poliposis colónica familiar / Intra-abdominal desmoid tumor mimicking a pancreatic tumor in a patient with familial colonic polyposis

IntroducciónLa fibromatosis es una proliferación fibroblástica benigna con crecimiento infiltrativo local. Se clasifica en una forma superficial y en una forma profunda, también denominada tumor desmoide (TD). Esta última puede presentarse en forma esporádica o asociada a la poliposis adenomatosa familiar y síndrome de Gardner. La presentación pancreática es excepcional y sólo existen 8 casos descritos en la literatura médica.Observación clínicaMujer de 29 años con antecedentes de PCF y 2 lesiones pancreáticas. En la pieza quirúrgica se observaron 2 lesiones mal delimitadas en el páncreas con infiltración de órganos vecinos. Histológicamente, correspondían a una proliferación de estirpe mesenquimatosa de patrón fusocelular sin atipias citológicas, que se diagnosticaron de TD.DiscusiónLa etiología de la fibromatosis es desconocida. En pacientes con PCF la localización más habitual de los TD es intraabdominal siendo inusual la presentación pancreática. Esto plantea el diagnóstico diferencial con otras neoplasias del páncreas(AU)

IntroductionFibromatosis 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.Case reportWe 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.DiscussionThe 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(AU)

[Intra-abdominal desmoid tumor mimicking a pancreatic tumor in a patient with familial colonic polyposis]. / Tumor desmoide intraabdominal simulando una tumoración pancreática en una paciente con poliposis colónica familiar.

INTRODUCTION: 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. CASE REPORT: 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. DISCUSSION: 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.