Primary Pancreatic-Type Acinar Cell Carcinoma of Jejunum Arising from Ectopic Pancreas with Synchronous Metastasis to Left Adrenal Gland

Abstract

Background: Pure pancreatic-type ACC outside the pancreas is extremely rare. Methods: We presented a very rare case of primary ACC of the pancreatic-type involving jejunum with a synchronous metastasis to left adrenal gland arising in well-developed ectopic pancreas which had fully developed pancreatic acini, islets of Langerhans, ductal structures, pancreatic cancer precursor lesions and a well-formed minor papilla that opens to the mucosal surface of jejunum. We also reviewed all 21 cases of pure pancreatic-type ACC outside the pancreas in the literature. Results: The pre-operative diagnosis of pancreatic-type ACC outside the pancreas is challenging and difficult based on imaging studies or on small biopsies. Only 2 of 14 cases, which reported a pre-operative diagnosis, were correctly diagnosed as pancreatic-type ACC on liver biopsy. Patients who presented with unresectable or stage IV disease had mean overall survival of 13.5 (standard deviation *SD+, 4.2) months, which was shorter than those who underwent surgical resection with curative intent (mean: 54.3, *SD, 9.1+ months, P = 0.008). Conclusions: The pre-operative diagnosis of pancreatic-type ACC outside the pancreas is OBM Hepatology and Gastroenterology 2019; 3(4), doi:10.21926/obm.hg.1904038 Page 2/13 challenging. Early detection and surgical resection may prolong survival in these patients.