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

World Journal of Surgical Oncology

Review

Feng Xu¹  Changjun Jia¹  Chaoliu Dai¹  Xianmin Bu¹  Baoling Tian²  Xinlu Wang³ 

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

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

DOI  :  10.1186/1477-7819-12-103

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

来源: Springer

【 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】

Unknown   
© Jia et al.; licensee BioMed Central Ltd. 2014. This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited.