: 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.
The Role of the Surgical Pathologist in the Recognition of Hereditary Neuroendocrine Tumors
La Rosa S.
2026-01-01
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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