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The Desmoid Tumor Research Foundation

September 2017

Desmoid type fibromatosis: A case report with an unusual etiology

Author Information

Jafri SF(1), Obaisi O(2), Vergara GG(3), Cates J(4), Singh J(5), Feeback J(6), Yandrapu H(7)

(1)Gastroenterology, Research Medical Center, Kansas City, MO 64132, United States.(2)School of Medicine, University of Missouri in Kansas City, Kansas City, MO 64108, United States.(3)Pathology, Research Medical Center, Kansas City, MO 64132, United States.(4)General and Vascular Surgery, Research Medical Center, Kansas City, MO 64132, United States.(5)Hematology/Oncology, Research Medical Center, Kansas City, MO 64132, United States.(6)Division Director of Clinical Research, Research Medical Center, Kansas City, MO 64132, United States.(7)Internal Medicine Department of Research Medical Center and Research Department of Kansas City Veterans Affairs Medical Center, Kansas City, MO 64132, United States.

Publication

World J Gastrointest Oncol

Summary

Desmoid type fibromatosis (DTF) is a rare, locally invasive, non-metastasizing soft tissue tumor. We report an interesting case of DTF involving the pancreatic head of a 54-year-old woman. She presented with intermittent dysphagia and significant weight loss within a 3-mo period. Laboratory findings showed mild elevation of transaminases, significant elevation of alkaline phosphatase and direct hyperbilirubinemia, indicating obstructive jaundice. Computerized tomography of the abdomen revealed a mass in the head of the pancreas, dilated common bile duct, and dilated pancreatic duct. Endoscopic retrograde cholangiopancreatography and endoscopic ultrasound showed a large hypoechoic mass in the head of the pancreas causing extrahepatic biliary obstruction and pancreatic ductal dilation. The patient underwent a successful partial pancreatico-duodenectomy and cholecystectomy. She received no additional therapy after surgery, and liver function tests were normalized within nine days after surgery. Currently, surgical resection is the recommended first line treatment. The patient will be followed for any recurrence.

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