2039 Invasive "Intracholecystic Papillary-Tubular Neoplasm" (ICPN) Presenting as Hemobilia in a Jehovah's Witness Patient

Abstract

INTRODUCTION: Hemobilia is a rare cause of upper gastrointestinal bleeding, which requires high index of suspicion for diagnosis. In various case series, hemobilia due to neoplastic disease of the hepatobiliary system ranges from 6% - 15%. ICPNs are rare neoplasms of the gallbladder that are usually detected incidentally. This is a case of a Jehovah’s Witness patient with severe symptomatic anemia secondary to hemobilia due to invasive ICPN. CASE DESCRIPTION/METHODS: 71-year-old Jehovah’s Witness patient with a 4 month history of anemia, suspected to be due to multiple small intestine angioectasias seen on prior enteroscopies, presented with a 3 day history of fatigue, dyspnea on exertion, and melena. Patient’s hemoglobin/hematocrit at presentation was 6.4/23.4. She underwent gastroduodenoscopy which showed blood in the second part of the duodenum which prompted a side viewing duodenoscopy examination which showed blood oozing from the ampulla (Figure 1). Abdominal MRI showed markedly distended gallbladder with polypoid appearing wall thickness and blood (Figure 2). Patient received iron infusions to improve hemoglobin and hematocrit. She then underwent open cholecystectomy, and intraoperatively the surgeons did not appreciate any mass lesion in the gallbladder. Surgical pathology found invasive mucinous adenocarcinoma (1.0 cm) arising from a large (14.5 cm) ICPN with high-grade dysplasia (Figures 3a,b). The tumor invaded the muscularis propria and had negative cystic duct margins for carcinoma or dysplasia. Two lymph nodes were positive for malignancy. It was staged as III-b and pT1b, pN1, pMx. After discussion in the tumor board, she was recommended to have surveillance imaging in 3 months because her comorbidities precluded chemotherapy. DISCUSSION: To the best of our knowledge this is the only reported case of ICPN presenting as hemobilia. Residual changes of ICPN are seen in only 6.4% of the patients with invasive GB carcinomas. The presence of angioectasias in the small intestine acted as a red herring in this patient. The fortuitous timing of endoscopy to visualize bleeding from the ampulla clinched the diagnosis of hemobilia. The presence of blood in the gallbladder made it difficult to identify the mass lesion which was eventually confirmed on surgical pathology. Hemobilia should always in the differential diagnosis for patients presenting with upper GI bleed and should be actively investigated when no obvious bleeding lesions are noted from the gastric or intestinal mucosa.