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Peer-reviewedCC BY 4.0Advances in Anatomic Pathology

The Role of the Surgical Pathologist in the Recognition of Hereditary Neuroendocrine Tumors

  • Silvia Uccella1,2
  • Claudio Luchini3,4
  • Atsuko Kasajima5
  • Günter Klöppel5
  • Stefano La Rosa6,7,8
8 affiliations
  1. 1Departments of Biomedical Sciences, Humanitas University, Milan
  2. 2Pathology Service, Istituti di Ricovero e Cura a Carattere Scientifico, Humanitas Research Hospital, Milan
  3. 3Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona
  4. 4ARC-NET Research Center, University of Verona, Verona
  5. 5Pathology, TUM School of Medicine and Health, Technical University Munich, Munich, Germany
  6. 6Medicine and Technological Innovation, Unit of Pathology
  7. 7Medicine and Technological Innovation, Hereditary Cancer Research Center, University of Insubria
  8. 8Unit of Pathology, University Hospital (ASST Sette Laghi), Varese, Italy
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Abstract

About 10% of neuroendocrine tumors (NETs) can occur in the context of several different hereditary tumor syndromes, and, in a not negligible number of cases, their diagnosis precedes the clinical identification of the hereditary syndrome. In this context, the role of a pathologist can be crucial in identifying these sentinel cases. Indeed, early recognition of inherited NETs has significant clinical value, both for guiding therapeutic strategies and for managing at-risk family members. Modern pathologists play a central role not only in the accurate diagnosis and subtyping of NETs but also in recognizing specific morphologic features that may point to a germline etiology. When morphology does not show peculiar features suggestive of a hereditary context, integrating morphology with clinical context may, however, help identify appropriate genetic testing. In the present paper, we provide an organ-based overview of the most important morphologic and immunohistochemical features, along with their molecular correlates, of pituitary, parathyroid, thoracic, and digestive NETs arising in the context of hereditary tumor syndromes.

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Cite

BibTeXRIS
BibTeX
@article{uccella2026role,
  title = {{The Role of the Surgical Pathologist in the Recognition of Hereditary Neuroendocrine Tumors}},
  author = {Silvia Uccella and Claudio Luchini and Atsuko Kasajima and Günter Klöppel and Stefano La Rosa},
  journal = {Advances in Anatomic Pathology},
  year = {2026},
  month = sep,
  doi = {10.1097/pap.0000000000000551},
  url = {https://doi.org/10.1097/pap.0000000000000551},
  note = {CC BY 4.0},
}
RIS
TY  - JOUR
TI  - The Role of the Surgical Pathologist in the Recognition of Hereditary Neuroendocrine Tumors
AU  - Silvia Uccella
AU  - Claudio Luchini
AU  - Atsuko Kasajima
AU  - Günter Klöppel
AU  - Stefano La Rosa
PY  - 2026
DA  - 2026/09/10
JO  - Advances in Anatomic Pathology
DO  - 10.1097/pap.0000000000000551
UR  - https://doi.org/10.1097/pap.0000000000000551
AB  - About 10% of neuroendocrine tumors (NETs) can occur in the context of several different hereditary tumor syndromes, and, in a not negligible number of cases, their diagnosis precedes the clinical identification of the hereditary syndrome. In this context, the role of a pathologist can be crucial in identifying these sentinel cases. Indeed, early recognition of inherited NETs has significant clinical value, both for guiding therapeutic strategies and for managing at-risk family members. Modern pathologists play a central role not only in the accurate diagnosis and subtyping of NETs but also in recognizing specific morphologic features that may point to a germline etiology. When morphology does not show peculiar features suggestive of a hereditary context, integrating morphology with clinical context may, however, help identify appropriate genetic testing. In the present paper, we provide an organ-based overview of the most important morphologic and immunohistochemical features, along with their molecular correlates, of pituitary, parathyroid, thoracic, and digestive NETs arising in the context of hereditary tumor syndromes.
SN  - 1072-4109
SN  - 1533-4031
ER  -