Jia Changjun, Tian Baoling, Dai Chaoliu, Wang Xinlu, Bu Xianmin, Xu Feng
Department of General Surgery, Shengjing Hospital, China Medical University, 36 Sanhao Street, Shenyang 110004, China.
World J Surg Oncol. 2014 Apr 22;12:103. doi: 10.1186/1477-7819-12-103.
Desmoid-type fibromatosis (DTF) is an uncommon nonmetastatic fibrous neoplasm. Sporadic intraperitoneal DTF is rarely described in current literature. We herein report a case of DTF of unknown cause involving the pancreatic head. A 41-year-old man presented with recurrent epigastric pain and weight loss. An abdominal computed tomography scan showed a well-delineated solid cystic mass inside the pancreatic head. Pylorus-preserving pancreaticoduodenectomy was performed due to the patient's debilitating symptoms and suspected malignancy. The pathological examination revealed massive fibroblastic proliferation arising from the musculoaponeurotic tissues, consistent with a diagnosis of DTF. Immunohistochemical phenotyping determined positive immunoreactivity to vimentin and β-catenin, but negative immunoreactivity to smooth muscle actin, CD117, CD34, or S-100, confirming the diagnosis of DTF. No local recurrence or distant metastasis was found during a 24-month follow-up. Radical resection is recommended as first-line treatment for pancreatic DTF. Long-term follow-up studies are required to establish the prognosis of pancreatic DTF.
韧带样型纤维瘤病（DTF）是一种罕见的非转移性纤维性肿瘤。目前文献中很少描述散发性腹膜内DTF。我们在此报告一例病因不明的累及胰头的DTF病例。一名41岁男性出现反复上腹部疼痛和体重减轻。腹部计算机断层扫描显示胰头内有一个边界清晰的实性囊性肿块。由于患者症状严重且怀疑为恶性肿瘤，遂行保留幽门的胰十二指肠切除术。病理检查显示肌筋膜组织出现大量成纤维细胞增生，符合DTF的诊断。免疫组织化学表型分析显示波形蛋白和β-连环蛋白免疫反应阳性，但平滑肌肌动蛋白、CD117、CD34或S-100免疫反应阴性，证实了DTF的诊断。在24个月的随访中未发现局部复发或远处转移。根治性切除建议作为胰腺DTF的一线治疗方法。需要进行长期随访研究以确定胰腺DTF的预后。
