Poorly differentiated neuroendocrine carcinomas of the pancreas: a clinicopathologic analysis of 44 cases. Academic Article uri icon

Overview

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.

authors

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

publication date

  • April 1, 2014

Research

keywords

  • Carcinoma, Neuroendocrine
  • Pancreatic Neoplasms

Identity

PubMed Central ID

  • PMC3977000

Scopus Document Identifier

  • 84897027158

Digital Object Identifier (DOI)

  • 10.1097/PAS.0000000000000169

PubMed ID

  • 24503751

Additional Document Info

volume

  • 38

issue

  • 4