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Review
. 2022 Feb;480(2):247-257.
doi: 10.1007/s00428-021-03211-5. Epub 2021 Oct 13.

Neuroendocrine neoplasms of the pancreas: diagnosis and pitfalls

Björn Konukiewitz  1 , Moritz Jesinghaus  2 , Atsuko Kasajima  3 , Günter Klöppel  3
Affiliations
Review

Neuroendocrine neoplasms of the pancreas: diagnosis and pitfalls

Björn Konukiewitz et al. Virchows Arch. 2022 Feb.

Abstract

Common to neuroendocrine neoplasms of the pancreas is their expression of synaptophysin, chromogranin A, and/or INSM1. They differ, however, in their histological differentiation and molecular profile. Three groups can be distinguished: well-differentiated neuroendocrine neoplasms (neuroendocrine tumors), poorly differentiated neuroendocrine neoplasms (neuroendocrine carcinomas), and mixed neuroendocrine-non-neuroendocrine neoplasms. However, the expression of synaptophysin and, to a lesser extent, also chromogranin A is not restricted to the neuroendocrine neoplasms, but may also be in a subset of non-neuroendocrine epithelial and non-epithelial neoplasms. This review provides the essential criteria for the diagnosis of pancreatic neuroendocrine neoplasms including diagnostic clues for the distinction of high-grade neuroendocrine tumors from neuroendocrine carcinomas and an algorithm avoiding diagnostic pitfalls in the delineation of non-neuroendocrine neoplasms with neuroendocrine features from pancreatic neuroendocrine neoplasms.

Keywords: Diagnosis; Histology; Immunohistology; Pancreatic neuroendocrine neoplasms; Pitfalls.

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Conflict of interest statement

The authors declare no competing interests.

Figures

Fig. 1
Fig. 1
Neuroendocrine tumors with solid (A) and trabecular (B) growth patterns
Fig. 2
Fig. 2
Liver metastasis of a neuroendocrine tumor G3 with an organoid growth pattern (A) showing monomorphous and round nuclei (B) and expression of synaptophysin (C) and Ki67 (index 25%) (D)
Fig. 3
Fig. 3
Neuroendocrine carcinoma, large cell type: solid cell clusters with pleomorphic nuclei showing a prominent nucleolus (A), and an overexpression of p53 (B). Fine-needle aspiration cytology specimens with matching expression of synaptophysin (C) and INSM1 (D)
Fig. 4
Fig. 4
Diagnostic algorithm for the differential diagnosis of synaptophysin expressing pancreatic neoplasms
Fig. 5
Fig. 5
Mixed acinar-neuroendocrine carcinoma with diffuse expression of synaptophysin (A) and trypsin (B)
Fig. 6
Fig. 6
Pancreatic SMARCB1 deficient rhabdoid neoplasm (A) with expression of synaptophysin (B), chromogranin A (C), and expression loss of INI1 (D)
See this image and copyright information in PMC

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