SHM Converge Scientific Abstract ePoster Gallery-A Gut-Wrenching Battle Within: An Unusual Case of Pancreatic Desmoid Fibromatosis

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This case report describes a rare pancreatic desmoid-type fibromatosis (DTF) in a 35-year-old woman with a history of laparoscopic sleeve gastrectomy. Desmoid tumors are uncommon soft tissue tumors that do not metastasize but can grow aggressively, invade nearby structures, and recur.<br /><br />The patient first presented with abdominal pain, vomiting, and poor oral intake. Initial labs were normal, but CT imaging showed a large pancreatic mass measuring about 9.4 x 10.5 cm, surrounding nearby vessels. A diagnostic laparoscopy and biopsy confirmed desmoid fibromatosis based on spindle-cell/myofibroblastic histology and beta-catenin positivity. Because the tumor was too extensive for surgery or radiotherapy, she began systemic treatment with nirogacestat and was discharged stable.<br /><br />Nine months later, her symptoms worsened and repeat CT showed tumor progression to 13 x 10 x 9.7 cm, with encasement of the celiac artery, splenic vein obstruction, portal vein narrowing, and severe stomach compression. Endoscopic ultrasound with fine needle aspiration again confirmed pancreatic DTF. Due to poor nutrition and inability to eat adequately, a jejunostomy tube was placed for enteral feeding. Her therapy was switched to liposomal doxorubicin, which she tolerated well. Follow-up MRI three months later showed a positive treatment response.<br /><br />The discussion notes that pancreatic DTF is very rare and may be associated with prior surgery, trauma, hormonal influences, or genetic factors. In this case, prior sleeve gastrectomy may have contributed. Management depends on tumor size, extent, and patient factors, and often requires a multidisciplinary approach. This case highlights the importance of individualized treatment for rare, locally invasive pancreatic desmoid tumors.

Asset Subtitle

Ahmed Qaedi

Meta Tag

Author List Ahmed Qaedi, Ali Hachem, Farrah Ibrahim

Category Clinical Vignettes

Concept Desmoid-Type Fibromatosis

Concept Pancreatic Desmoid Fibromatosis

Concept Locally Invasive Growth

Concept Mass Effect

Concept Spindle Cell Proliferation

Distinguished Non-Finalist

Presenter Organization University of Alabama at Birmingham, Huntsville Regional Medical Campus

Presenting Author Ahmed Qaedi

Track Adult

Keywords

pancreatic desmoid-type fibromatosis

desmoid tumor

rare pancreatic mass

beta-catenin positivity

laparoscopic sleeve gastrectomy

spindle-cell histology

nirogacestat

liposomal doxorubicin

jejunostomy tube

multidisciplinary management

Desmoid-Type Fibromatosis

Pancreatic Desmoid Fibromatosis

Locally Invasive Growth

Mass Effect

Spindle Cell Proliferation