Poorly differentiated neuroendocrine carcinomas of the pancreas: A clinicopathologic analysis of 44 cases

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

Research output: Contribution to journalArticle

101 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)437-447
Number of pages11
JournalAmerican Journal of Surgical Pathology
Volume38
Issue number4
DOIs
StatePublished - 2014

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Neuroendocrine Carcinoma
Pancreas
Large Cell Carcinoma
Neuroendocrine Tumors
Acinar Cell Carcinoma
Neoplasms
Neuroendocrine Cells
Small Cell Carcinoma
Survival
Neoplasm Metastasis
Differentiation Antigens
Diagnostic Errors
Adenocarcinoma
Survival Rate

Keywords

  • Carcinoma
  • High grade
  • Large cell
  • Neuroendocrine
  • Pancreas
  • Poorly differentiated
  • Small cell

ASJC Scopus subject areas

  • Anatomy
  • Pathology and Forensic Medicine
  • Surgery

Cite this

Basturk, O., Tang, L., Hruban, R. H., Adsay, V., Yang, Z., Krasinskas, A. M., ... Klimstra, D. S. (2014). Poorly differentiated neuroendocrine carcinomas of the pancreas: A clinicopathologic analysis of 44 cases. American Journal of Surgical Pathology, 38(4), 437-447. https://doi.org/10.1097/PAS.0000000000000169

Poorly differentiated neuroendocrine carcinomas of the pancreas : A clinicopathologic analysis of 44 cases. / 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.

In: American Journal of Surgical Pathology, Vol. 38, No. 4, 2014, p. 437-447.

Research output: Contribution to journalArticle

Basturk, O, Tang, L, Hruban, RH, Adsay, V, Yang, Z, Krasinskas, AM, Vakiani, E, La Rosa, S, Jang, KT, Frankel, WL, Liu, X, Zhang, L, Giordano, TJ, Bellizzi, AM, Chen, JH, Shi, C, Allen, P, Reidy, DL, Wolfgang, CL, Saka, B, Rezaee, N, Deshpande, V & Klimstra, DS 2014, 'Poorly differentiated neuroendocrine carcinomas of the pancreas: A clinicopathologic analysis of 44 cases', American Journal of Surgical Pathology, vol. 38, no. 4, pp. 437-447. https://doi.org/10.1097/PAS.0000000000000169
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. / Poorly differentiated neuroendocrine carcinomas of the pancreas : A clinicopathologic analysis of 44 cases. In: American Journal of Surgical Pathology. 2014 ; Vol. 38, No. 4. pp. 437-447.
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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.",
keywords = "Carcinoma, High grade, Large cell, Neuroendocrine, Pancreas, Poorly differentiated, Small cell",
author = "Olca Basturk and Laura Tang and Hruban, {Ralph H.} and Volkan Adsay and Zhaohai Yang and Krasinskas, {Alyssa M.} and Efsevia Vakiani and {La Rosa}, Stefano and Jang, {Kee Taek} and Frankel, {Wendy L.} and Xiuli Liu and Lizhi Zhang and Giordano, {Thomas J.} and Bellizzi, {Andrew M.} and Chen, {Jey Hsin} and Chanjuan Shi and Peter Allen and Reidy, {Diane L.} and Wolfgang, {Christopher L.} and Burcu Saka and Neda Rezaee and Vikram Deshpande and Klimstra, {David S.}",
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TY - JOUR

T1 - Poorly differentiated neuroendocrine carcinomas of the pancreas

T2 - A clinicopathologic analysis of 44 cases

AU - Basturk, Olca

AU - Tang, Laura

AU - Hruban, Ralph H.

AU - Adsay, Volkan

AU - Yang, Zhaohai

AU - Krasinskas, Alyssa M.

AU - Vakiani, Efsevia

AU - La Rosa, Stefano

AU - Jang, Kee Taek

AU - Frankel, Wendy L.

AU - Liu, Xiuli

AU - Zhang, Lizhi

AU - Giordano, Thomas J.

AU - Bellizzi, Andrew M.

AU - Chen, Jey Hsin

AU - Shi, Chanjuan

AU - Allen, Peter

AU - Reidy, Diane L.

AU - Wolfgang, Christopher L.

AU - Saka, Burcu

AU - Rezaee, Neda

AU - Deshpande, Vikram

AU - Klimstra, David S.

PY - 2014

Y1 - 2014

N2 - 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.

AB - 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.

KW - Carcinoma

KW - High grade

KW - Large cell

KW - Neuroendocrine

KW - Pancreas

KW - Poorly differentiated

KW - Small cell

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DO - 10.1097/PAS.0000000000000169

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