P2986 - Solid Pseudopapillary Neoplasm in Pregnancy: A Multidisciplinary Approach

Raakhi Menon, DO¹, Anni Chowdhury, DO¹, Jaison S. John, MD¹, Arsalan Saleem, MD¹, John Walker, MD¹, Gayle Olson Koutrouvelis, MD, MPH¹, Aspen Stuart, MD¹, Suimin Qiu, MD, PhD², Palawinnage Muthukumarana, MD¹, Sreeram Parupudi, MD^1
1University of Texas Medical Branch, Galveston, TX; ²UTMB, Galveston, TX

Introduction: Solid pseudopapillary neoplasm (SPN) is a rare, low-grade malignant pancreatic tumor with a strong predilection for women in their 3^rd decade. Parenchyma-preserving surgery offers good prognosis. Expression of progesterone receptors is its hallmark, which may explain progression in pregnancy. It is usually detected on routine prenatal ultrasound (US). Unless impending rupture, surgery is deferred to postpartum. We describe here a multidisciplinary management of a complex case of SPN diagnosed during pregnancy.



Case Description/Methods: A 28-year-old female in 3^rd trimester was admitted due to concern for intrahepatic cholestasis (ICP) of pregnancy. US showed normal biliary system and a large right adnexal mass, which appeared as a complex cystic lesion on MRI. Worsening liver enzymes led to the delivery of a healthy baby at term via cesarian section. During surgery, a large intra-abdominal mass was noted extending towards the right upper quadrant, but resection was deferred. CT abdomen showed a 14-cm heterogeneous hypodense mass arising from the uncinate process of pancreas. Parenchyma-preserving partial pancreatectomy was performed 5 weeks postpartum, confirming the histology. Injury to superior mesenteric vein from surgery was repaired, but associated bowel edema necessitated staged closure of the abdomen. The postoperative course was stormy, requiring drainage of infected collections and coil-embolization of the gastroduodenal artery for internal bleeding. During her slow recovery, a pancreatic leak was suspected due to high amylase in drain output. Endoscopic retrograde cholangiopancreatography failed due to severe erosive duodenitis, where a small duodenal fistula was noted below the ampulla. Paraduodenal pooling of contrast without opacification of the bile duct was observed on fistulogram. Percutaneous cholangiography showed a dilated bile duct with complete distal occlusion. Following initial external drainage, recanalization of the bile duct was performed, targeting an open snare deployed in the duodenum making a neo-tract facilitating an internal-external (IE) drainage. The IE stent was then internalized with a covered biliary metal stent for future endoscopic management.

Discussion: In the absence of guidelines, management of complicated masses like SPN during pregnancy must be tailored to each patient, balancing the need to prevent catastrophic rupture with a favorable gestational outcome. Successful care of this rare tumor requires a multidisciplinary strategy implemented in stages.



Disclosures:


Raakhi Menon, DO¹, Anni Chowdhury, DO¹, Jaison S. John, MD¹, Arsalan Saleem, MD¹, John Walker, MD¹, Gayle Olson Koutrouvelis, MD, MPH¹, Aspen Stuart, MD¹, Suimin Qiu, MD, PhD², Palawinnage Muthukumarana, MD¹, Sreeram Parupudi, MD¹. P2986 - Solid Pseudopapillary Neoplasm in Pregnancy: A Multidisciplinary Approach, ACG 2023 Annual Scientific Meeting Abstracts. Vancouver, BC, Canada: American College of Gastroenterology.