腹腔内韧带样型纤维瘤病1例

Intraabdominal Desmoid Fibromatosis: A Case Report

摘要: 韧带样型纤维瘤病是一种罕见肿瘤，本文报道1例发生在成人的腹腔内韧带样型纤维瘤病：患者，女，49岁，阵发性上腹痛20天，就诊于我院，行增强MRI示：胰腺胃小弯间隙占位，分界欠清，恶性肿瘤可能。手术切除后病理结果见：星状、梭形细胞结节状增生，间质局部玻璃样与粘液变性，累及胰腺、横结肠肠壁。免疫组化染色示：β-Catenin (+)。诊断为腹腔内韧带样型纤维瘤病。韧带样型纤维瘤病罕见，术前诊断与诊疗计划的制定对该病的整体治疗意义重大，需要提高对该疾病的认识。

Abstract: Desmoid-type fibromatosis is a rare tumor. This article reports a case of intra-abdominal des moid-type fibromatosis in an adult. The patient, a 49-year-old female, presented with paroxysmal epigastric pain for 20 days and was admitted to our hospital. Contrast-enhanced MRI revealed a mass in the pancreatic-gastric lesser curvature space with ill-defined margins, suggesting a possible malignancy. Post-surgical resection, pathological examination showed nodular hyperplasia of stellate and spindle-shaped cells, with focal stromal hyalinization and mucoid degeneration, infiltrating the pancreas and the wall of the transverse colon. Immunohistochemical staining was positive for β-Catenin. The diagnosis was intra-abdominal desmoid-type fibromatosis. Desmoid-type fibromatosis is rare, and its preoperative diagnosis and management planning are crucial for the overall treatment of this disease, highlighting the need to enhance recognition of this condition.