High-Grade Neuroendocrine Carcinomas of the Pancreas: A Clinicopathologic Analysis of 60 Cases
Olca Basturk, Ralph Hruban, Volkan Adsay, Alyssa Krasinskas, Kee-Taek Jang, Xiuli Liu, Efsevia Vakiani, Lizhi Zhang, Wendy Frankel, Thomas Giordano, Andrew Bellizzi, Jey-Hsin Chen, Vikram Deshpande, Zhaohai Yang, David S Klimstra. Memorial Sloan-Kettering Cancer Center, New York, NY; Johns Hopkins University, Baltimore, MD; Emory University, Atlanta, GA; University of Pittsburgh, Pittsburgh, PA; Samsung MC, Seoul, Korea; Cleveland Clinic, Cleveland, OH; Mayo Clinic, Rochester, MN; Ohio State University, Colombus, OH; University of Michigan, Ann Arbor, MI; Brigham and Women's Hospital, Boston, MD; Indiana University, Indianapolis, IN; Penn State Hershey MC, Hershey, PA
Background: In the pancreas, data regarding the types and significance of high-grade neuroendocrine carcinoma (HGNEC) has been very limited.
Design: Ninety-three cases, biopsied or surgically resected at 12 different institutions from 1988-2012, that had been diagnosed as HGNEC were reassessed with the morphologic criteria employed in the lung as well as IHC labeling with NE (chromogranin/synaptophysin) and acinar (trypsin/chymotrypsin) markers. Thirty-three were excluded, most being reclassified as acinar cell carcinoma (ACC). Remaining 60 were assessed as HGNEC for clinicopathologic features and survival outcomes.
Results: The mean age of patients qualified as HGNEC was 58 (range, 27-84). M/F ratio was 1.2. Increased serum hormone levels were present in only 3 (5%) patients (insulin in 2, VIP in 1). Sixty percent of the tumors were localized in the head of the gland, 10% in the body and 30% in the tail. Median size was 4.5 cm (range, 2.3-20). The majority (92%) of the tumors was pure NEC, 38% of which were small cell type. There was an associated adenocarcinoma component in 4, and an IPMN in 1. The incidence of vascular and perineural invasion were 71% and 62%, respectively. In 55%, one or more, usually the retroperitoneal, surgical margin was positive. Sixty-three percent of the patients had metastatic disease at presentation and an additional 28% subsequently developed metastases, usually to the regional lymph nodes and liver. Follow-up (F/U) information was available in 37 (62%) patients. 26 died of disease, with a median survival of 11 mos (range, 0-77); 11 patients were alive with disease, with a median F/U of 17 mos (range, 0-50). The median survival for all cases was 15.1 mos, with 5-yr survival of 17%.
Conclusions: Our data demonstrates that ACC often get misdiagnosed as HGNEC and strongly suggests that thorough immunohistochemical evaluation be performed before HGNEC diagnosis is rendered. HGNECs of the pancreas is a highly aggressive neoplasm, with frequent metastases and poor survival. Most patients die within less than a year. About 40% of the tumors are of small cell type and the majority of the tumors are non-functional.
Tuesday, March 5, 2013 9:30 AM
Poster Session III # 206, Tuesday Morning