Idiopathic desmoid-type fibromatosis of the pancreatic head: case report and literature review

World Journal of Surgical Oncology

Feng Xu³  Xianmin Bu³  Xinlu Wang¹  Chaoliu Dai³  Baoling Tian²  Changjun Jia³ 

[1] Department of Ultrasound Medicine, Shengjing Hospital, China Medical University, 36 Sanhao Street, Shenyang 110004, China;Department of Pathology, Shengjing Hospital, China Medical University, 36 Sanhao Street, Shenyang 110004, China;Department of General Surgery, Shengjing Hospital, China Medical University, 36 Sanhao Street, Shenyang 110004, China

关键词: Immunohistochemistry;    Musculoaponeurotic tissues;    Pancreatic head;    Aggressive fibromatosis;    Desmoid tumor;    Desmoid-type fibromatosis;   

Others  :  819862
DOI  :  10.1186/1477-7819-12-103

 received in 2013-05-26, accepted in 2014-04-07,  发布年份 2014

【 摘 要 】

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.

【 授权许可】

   
2014 Jia et al.; licensee BioMed Central Ltd.

【 图 表 】

Figure 1.

Figure 2.

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