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Pancreas

Neuroendocrine tumors

Neuroendocrine carcinoma, NOS

Reviewer: Deepali Jain, M.D. (see Reviewers page)
Revised: 29 November 2012, last major update August 2012
Copyright: (c) 2001-2012, PathologyOutlines.com, Inc.

General
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● Poorly differentiated, high grade malignant neoplasm, composed of small, intermediate or large cells, variable organoid features resembling neutroendocrine tumor, diffusely expressing neuroendocrine markers (diffuse staining for synaptophysin; faint or focal staining for chromogranin A), with marked nuclear atypia, multifocal necrosis and a high number of mitoses (> 20 per 10 HPF) (WHO)
● High grade (G3) is defined by proliferation fraction and histology
● Includes small cell carcinoma, large cell (neuro)endocrine carcinoma, poorly differentiated (neuro)endocrine carcinoma
● Genetically dissimilar from well differentiated neuroendocrine tumors (Am J Surg Pathol 2012;36:173)

Clinical features
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● Represents less than 3% of pancreatic neuroendocrine tumors
● May produce ACTH
● Associated with hypercalcemia

Case reports
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● 62 year old man with tail tumor (World J Surg Oncol 2012;10:32)

Gross images
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Small cell carcinoma

Micro images
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Large number of poorly differentiated fusiform cells and focal necrosis


High mitotic rate

Small Cell Carcinoma
     
Various images


Solid-diffuse pattern

 
Left: NSE+; right: scattered somatostatin-immunoreactive cells

Cytology images
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Metastatic pulmonary small cell carcinoma to pancreas

Differential diagnosis
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● Acinar cell carcinoma: immunoreactive for trypsin or chymotrypsin, note: may be positive for neuroendocrine markers
● Ductal adenocarcinoma, poorlly differentiated: negative for neuroendocrine markers
● Metastatic carcinoma: clinical history needed
● NET G1/G2: lower mitotic rate, well differentiated histology
● PNET: young patients

End of Pancreas > Neuroendocrine tumors > Neuroendocrine carcinoma, NOS

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