D0028 - Exceedingly Rare Coexistence of Metamorphosis of Tubulovillous Adenoma of the Ampulla to Adenocarcinoma with Small Bowel Gastrointestinal Stromal Tumor - Case Report and Review of the Literature
Venkata Vinod Kumar Matli, MD1, Gregory Wellman, MD1, Poornima Ramadas, MD2, Sudha Pandit, MD3, Qiang Cai, MD3, Gazi B. Zibari, MD4 1Christus Highland Medical Center, Shreveport, LA; 2LSU Health, Shreveport, LA; 3LSUHSC, Shreveport, LA; 4Willis Knighton Health System, Shreveport, LA
Introduction: This is a unique case of a patient with three tumors, which include one benign tumor, tubulo-villous adenoma of the ampulla of Vater (TVAoA), and two extremely rare malignant tumors, adenocarcinoma of the ampulla of Vater arising from TVAoA and gastrointestinal stromal tumor (GIST) involving the jejunum. Based on literature review, this is the first case of ampullary adenocarcinoma coexisting with GIST. Distal duodenal polyps are uncommon and have preponderance to occur in and around the ampulla of Vater. We report a case of a 77-year-old male who was admitted for painless obstructive jaundice with a 40-pound weight loss over a two-month period, and who was subsequently diagnosed with three tumors.
Case Description/Methods: A 77-year-old male was admitted for generalized weakness with associated weight loss of 40 pounds in the previous two months and was noted to have painless obstructive jaundice. Computed tomography (CT) of the abdomen and pelvis and magnetic resonance cholangiopancreatography were consistent with a polypoid mass at the level of the common bile duct (CBD) and ampulla of Vater with CBD dilatation. The same lesions were visualized by endoscopic retrograde cholangiopancreatography. Histopathology of endoscopic forceps biopsy showed tubulo-villous AoA (TVAoA). Histopathology of the surgical specimen of the resected ampulla showed adenocarcinoma arising from the TVAoA. Abdominal and pelvic CT also showed a coexisting heterogeneously enhancing, lobulated mass in the posterior pelvis originating from the jejunum. The patient underwent resection of the mass and jejunojejunal anastomosis. The histopathology of the resected mass confirmed it as a high-grade GIST.
Discussion: AoA can occur sporadically and in a familial inheritance pattern in the setting of FAPS. We emphasize screening and surveillance colonoscopy when one encounters AoA in upper endoscopy to check for FAPS. AoA is a premalignant lesion, particularly in the setting of FAPS, that carries a high risk of metamorphism to ampullary adenocarcinoma. Final diagnosis should be based on a histopathologic study of the surgically resected ampullary specimen, and not on endoscopic forceps biopsy.This case is unique because he had three tumors and rare coexistence, which included one premalignant TVAoA and two malignant tumors: ampullary adenocarcinoma arising from the TVAoA and coexisting jejunal GIST.
Figure: Figure 1A: CT abdomen and pelvis with contrast. Obstructed distal common bile duct due to a 9 mm polypoid intraluminal (pointed yellow arrows) lesion in the distal CBD. Figure 1B: CT of the abdomen and pelvis with contrast. Heterogeneously enhanced lobulated mass with punctate calcifications (pointed yellow arrows) in the posterior pelvis originating from the serosal surface of the pelvic small bowel. Figure 1C: Endoscopic retrograde cholangiopancreatography (ERCP) showed abnormal papilla with polypoid mass. Sphincterotomy and deep cannulation procedures were performed and confirmed by fluoroscopy. It showed common bile duct dilatation, and there was an abrupt cutoff at the distal aspect. Figure 1D: High-power photomicrograph showing high-grade glandular dysplasia (hematoxylin and eosin stain, 200 X original magnification) Figure 1E: A broad base ampullary mass with red fogerty cath was advanced from the cystic duct down through the ampulla to the duodenum. Figure 1F: Pedunculated GIST arising from the small bowel approximately 150 cm proximal to the ileocaecal valve.
Disclosures:
Venkata Vinod Kumar Matli indicated no relevant financial relationships.
Gregory Wellman indicated no relevant financial relationships.
Poornima Ramadas indicated no relevant financial relationships.
Sudha Pandit indicated no relevant financial relationships.
Qiang Cai indicated no relevant financial relationships.
Gazi Zibari indicated no relevant financial relationships.
Venkata Vinod Kumar Matli, MD1, Gregory Wellman, MD1, Poornima Ramadas, MD2, Sudha Pandit, MD3, Qiang Cai, MD3, Gazi B. Zibari, MD4. D0028 - Exceedingly Rare Coexistence of Metamorphosis of Tubulovillous Adenoma of the Ampulla to Adenocarcinoma with Small Bowel Gastrointestinal Stromal Tumor - Case Report and Review of the Literature, ACG 2022 Annual Scientific Meeting Abstracts. Charlotte, NC: American College of Gastroenterology.
