Poorly differentiated neuroendocrine carcinomas of the pancreas: A clinicopathologic analysis of 44 cases Journal Article
| Authors: | Basturk, O.; Tang, L.; Hruban, R. H.; Adsay, V.; Yang, Z.; Krasinskas, A. M.; Vakiani, E.; La Rosa, S.; Jang, K. T.; Frankel, W. L.; Liu, X.; Zhang, L.; Giordano, T. J.; Bellizzi, A. M.; Chen, J. H.; Shi, C.; Allen, P.; Reidy, D. L.; Wolfgang, C. L.; Saka, B.; Rezaee, N.; Deshpande, V.; Klimstra, D. S. |
| Article Title: | Poorly differentiated neuroendocrine carcinomas of the pancreas: A clinicopathologic analysis of 44 cases |
| 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. |
| Keywords: | pancreas; carcinoma; small cell; neuroendocrine; poorly differentiated; high grade; large cell |
| Journal Title: | American Journal of Surgical Pathology |
| Volume: | 38 |
| Issue: | 4 |
| ISSN: | 0147-5185 |
| Publisher: | Lippincott Williams & Wilkins |
| Date Published: | 2014-04-01 |
| Start Page: | 437 |
| End Page: | 447 |
| Language: | English |
| DOI: | 10.1097/pas.0000000000000169 |
| PROVIDER: | scopus |
| PMCID: | PMC3977000 |
| PUBMED: | 24503751 |
| DOI/URL: | |
| Notes: | Export Date: 1 May 2014 -- CODEN: AJSPD -- Source: Scopus |
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