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American Journal of Surgical Pathology:
doi: 10.1097/PAS.0000000000000169
Original Articles

Poorly Differentiated Neuroendocrine Carcinomas of the Pancreas: A Clinicopathologic Analysis of 44 Cases

Basturk, Olca MD*; Tang, Laura MD, PhD*; Hruban, Ralph H. MD; Adsay, Volkan MD; Yang, Zhaohai MD, PhD§; Krasinskas, Alyssa M. MD; Vakiani, Efsevia MD, PhD*; La Rosa, Stefano MD; Jang, Kee-Taek MD, PhD#; Frankel, Wendy L. MD**; Liu, Xiuli MD, PhD††; Zhang, Lizhi MD‡‡; Giordano, Thomas J. MD§§; Bellizzi, Andrew M. MD∥∥; Chen, Jey-Hsin MD, PhD¶¶; Shi, Chanjuan MD##; Allen, Peter MD***; Reidy, Diane L. MD†††; Wolfgang, Christopher L. MD, PhD†,‡‡‡; Saka, Burcu MD; Rezaee, Neda MD†,‡‡‡; Deshpande, Vikram MD§§§; Klimstra, David S. MD*

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Abstract

Background: In the pancreas, poorly differentiated neuroendocrine carcinomas include small cell carcinoma and large cell neuroendocrine carcinoma and are rare; data regarding their pathologic and clinical features are very limited.

Design: A total of 107 pancreatic resections originally diagnosed as poorly differentiated neuroendocrine carcinomas were reassessed using the classification and grading (mitotic rate/Ki67 index) criteria put forth by the World Health Organization in 2010 for the gastroenteropancreatic system. Immunohistochemical labeling for neuroendocrine and acinar differentiation markers was performed. Sixty-three cases were reclassified, mostly as well-differentiated neuroendocrine tumor (NET) or acinar cell carcinoma, and eliminated. The clinicopathologic features and survival of the remaining 44 poorly differentiated neuroendocrine carcinomas were further assessed.

Results: The mean patient age was 59 years (range, 21 to 82 y), and the male/female ratio was 1.4. Twenty-seven tumors were located in the head of the pancreas, 3 in the body, and 11 in the tail. The median tumor size was 4 cm (range, 2 to 18 cm). Twenty-seven tumors were large cell neuroendocrine carcinomas, and 17 were small cell carcinomas (mean mitotic rate, 37/10 and 51/10 HPF; mean Ki67 index, 66% and 75%, respectively). Eight tumors had combined components, mostly adenocarcinomas. In addition, 2 tumors had components of well-differentiated NET. Eighty-eight percent of the patients had nodal or distant metastatic disease at presentation, and an additional 7% developed metastases subsequently. Follow-up information was available for 43 patients; 33 died of disease, with a median survival of 11 months (range, 0 to 104 mo); 8 were alive with disease, with a median follow-up of 19.5 months (range, 0 to 71 mo). The 2- and 5-year survival rates were 22.5% and 16.1%, respectively.

Conclusions: Poorly differentiated neuroendocrine carcinoma of the pancreas is a highly aggressive neoplasm, with frequent metastases and poor survival. Most patients die within less than a year. Most (61%) are large cell neuroendocrine carcinomas. Well-differentiated NET and acinar cell carcinoma are often misdiagnosed as poorly differentiated neuroendocrine carcinoma, emphasizing that diagnostic criteria need to be clearly followed to ensure accurate diagnosis.

© 2014 by Lippincott Williams & Wilkins.

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