Journal article
POORLY DIFFERENTIATED NEUROENDOCRINE CARCINOMAS OF THE PANCREAS: A CLINICOPATHOLOGIC ANALYSIS OF 44 CASES
The American journal of surgical pathology, Vol.38(4), pp.437-447
04/2014
DOI: 10.1097/PAS.0000000000000169
PMCID: PMC3977000
PMID: 24503751
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.
Details
- Title: Subtitle
- POORLY DIFFERENTIATED NEUROENDOCRINE CARCINOMAS OF THE PANCREAS: A CLINICOPATHOLOGIC ANALYSIS OF 44 CASES
- Creators
- Olca Basturk - Departments of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NYLaura Tang - Departments of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NYRalph H Hruban - Departments of Pathology, The Sol Goldman Pancreatic Cancer Research Center, Johns Hopkins University, Baltimore, MDN. Volkan Adsay - Departments of Pathology, Emory University, Atlanta, GAZhaohai Yang - Departments of Pathology, Penn State Hershey MC, Hershey, PAAlyssa M Krasinskas - Departments of Pathology, University of Pittsburgh, Pittsburgh, PAEfsevia Vakiani - Departments of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NYStefano La Rosa - Departments of Pathology, Ospedale di Circolo, Varese, ItalyKee-Taek Jang - Departments of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, KoreaWendy L Frankel - Departments of Pathology, Ohio State University, Columbus, OHXiuli Liu - Departments of Pathology, Cleveland Clinic, Cleveland, OHLizhi Zhang - Departments of Pathology, Mayo Clinic, Rochester, MNThomas J Giordano - Departments of Pathology, University of Michigan, Ann Arbor, MIAndrew M Bellizzi - Departments of Pathology, University of Iowa Hospitals and Clinics, Iowa City, IAJey-Hsin Chen - Departments of Pathology, Indiana University, Indianapolis, INChanjuan Shi - Departments of Pathology, Vanderbilt University, Nashville, TNPeter Allen - Departments of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NYDiane L Reidy - Department of Oncology, Memorial Sloan-Kettering Cancer Center, New York, NYChristopher L Wolfgang - Departments of Pathology, The Sol Goldman Pancreatic Cancer Research Center, Johns Hopkins University, Baltimore, MDBurcu Saka - Departments of Pathology, Emory University, Atlanta, GANeda Rezaee - Departments of Pathology, The Sol Goldman Pancreatic Cancer Research Center, Johns Hopkins University, Baltimore, MDVikram Deshpande - Departments of Pathology, Massachusetts General Hospital, Boston, MADavid S Klimstra - Departments of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY
- Resource Type
- Journal article
- Publication Details
- The American journal of surgical pathology, Vol.38(4), pp.437-447
- DOI
- 10.1097/PAS.0000000000000169
- PMID
- 24503751
- PMCID
- PMC3977000
- NLM abbreviation
- Am J Surg Pathol
- ISSN
- 0147-5185
- eISSN
- 1532-0979
- Language
- English
- Date published
- 04/2014
- Academic Unit
- Pathology
- Record Identifier
- 9984047987202771
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