An Unusual Cause of Acute Pancreatitis in a Young Woman

Abstract

Question: A 27-year-old woman presented to the emergency department with intermittent epigastric pain radiating to the back of one week’s duration. The pain was dull and associated with nausea. Her medical and family history was unremarkable. She denied any alcohol or drug intake. There was no history of fever, yellowish discoloration of the sclera or urine, or weight loss. Upon arrival, she had mild tachycardia (heart rate: 103 beats per minute). Physical examination revealed tenderness in the epigastric and right upper quadrant of the abdomen. The Murphy’s sign was negative, and there were no peritoneal signs. The remainder of the physical examination was unremarkable. Laboratory studies showed an elevated serum aspartate aminotransferase level of 176 IU/l, alanine aminotransferase of 240 IU/l, gamma-glutamyl transpeptidase of 757 IU/L, alkaline phosphatase of 308 IU/l, total bilirubin of 0.6 mg/dL, lipase of 6,641 U/L, amylase of 667 U/L, and high sensitivity C-reactive protein of 3.416 mg/L; white blood cell count, serum bilirubin, triglyceride, and calcium levels were all within normal limits. Contrast-enhanced computed tomography (CT) of the abdomen showed marked peripancreatic fat stranding, no dilatation of the biliary tract or pancreatic duct, and no evidence of regional lymphadenopathy (Figure A). An endoscopic retrograde cholangiopancreatography (ERCP) was carried out to rule out biliary pancreatitis. Duodenoscopy showed a 3-cm pedunculated tumor with a smooth surface and soft texture at the Vater’s papilla (Figure B and C). Endoscopic ultrasonography (EUS) revealed the tumor as smooth-margined, homogenous, hypoechoic lesion arising from the submucosal layer (Figure D). What is your diagnosis of the lesion of the Vater’s papilla?A.CholedochocoeleB.Neuroendocrine tumor (NET)C.Gastrointestinal stromal tumor (GIST)D.Gangliocytic paraganglioma Look on page 621 for the answer and see the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image(s) to Practical Teaching Cases. The correct answer is D. Gangliocytic paraganglioma (GP) of the Vater’s papilla. The patient underwent surgical wedge resection of the tumor (Figure E). The histopathological examination showed the tumor located in the submucosa (Figure F, H&E, 40×). GP is typically composed of spindle cells, ganglion cells, and epithelioid cells with the appearance of neuroendocrine cells (Figure G, H&E, 200×; Figure H, H&E, 400×). There was no mitosis or necrosis in the tumor. Immunohistochemistry examination revealed that the neuroendocrine cells stained positive for CD56 and chromogranin A, and the spindle cells were immunoreactive for S100 protein. These findings confirmed the diagnosis of GP. Answer A is incorrect because choledochocele is an anechoic lesion on EUS. Gastrointestinal NETs are homogenously hypoechoic lesions arising from the second or third layer, sometimes with muscularis propria expansion (Answer B). The EUS imaging feature of a GIST is a hypoechoic mass arising from the muscularis propria, and larger GIST are sometimes composed of heterogeneous echo patterns and anechoic spaces (Answer C). However, the definitive diagnosis of the lesion requires histology. GP is a rare, generally benign neuroendocrine tumor. However, GP with regional lymph node involvement and distant metastasis have been reported.1Li B. Li Y. Tian X.Y. et al.Malignant gangliocytic paraganglioma of the duodenum with distant metastases and a lethal course.World J Gastroenterol. 2014; 20: 15454-15461Crossref PubMed Scopus (28) Google Scholar The most common site of the tumor is the second portion of the duodenum.2Okubo Y. Nemoto T. Wakayama M. et al.Gangliocytic paraganglioma: a multi-institutional retrospective study in Japan.BMC Cancer. 2015; 15: 269Crossref PubMed Scopus (28) Google Scholar, 3Okubo Y. Wakayama M. Nemoto T. et al.Literature survey on epidemiology and pathology of gangliocytic paraganglioma.BMC Cancer. 2011; 11: 187Crossref PubMed Scopus (74) Google Scholar Gastrointestinal bleeding—followed by abdominal pain and anemia—are the most common symptoms of the disease.3Okubo Y. Wakayama M. Nemoto T. et al.Literature survey on epidemiology and pathology of gangliocytic paraganglioma.BMC Cancer. 2011; 11: 187Crossref PubMed Scopus (74) Google Scholar GPs without distant metastasis are usually treated via endoscopic resection or pylorus-preserving pancreaticoduodenectomy.2Okubo Y. Nemoto T. Wakayama M. et al.Gangliocytic paraganglioma: a multi-institutional retrospective study in Japan.BMC Cancer. 2015; 15: 269Crossref PubMed Scopus (28) Google Scholar The patient recovered uneventfully and had no evidence of tumor recurrence after a surgical wedge resection of the duodenal GP at the one-year follow-up. In conclusion, GP may be an uncommon cause of acute pancreatitis. This rare and benign disease can be treated via endoscopic or surgical resection of the tumor. CME Exam 1: An Unusual Cause of Acute Pancreatitis in a Young WomanGastroenterologyVol. 157Issue 3Preview Full-Text PDF