Work

Erbliche neuroendokrine Neoplasien

Year: 2026

Type: article

Abstract: Hereditary neuroendocrine neoplasms (NENs) are the basis of over 10% of all neuroendocrine tumors. They result from germline mutations in tumor suppressor genes or proto-oncogenes.Compared to sporadic forms, hereditary NENs usually manifest at a younger age. Characteristic features include multifocal tumor development within a single organ or the synchronous occurrence of tumors in different endocrine tissues. Histologically, specific precursor lesions, such as endocrine hyperplasia or microtumors, can provide diagnostic clues. Furthermore, immunohistochemical analyses can directly detect the loss of gene products (e.g., SDHB, menin), serving as a guide for the mandatory molecular genetic germline analysis.This overview explains the clinical and histological signs that may indicate a familial background.

Source: Die Pathologie

DOI: 10.1007/s00292-026-01572-9

Authors: Aziz Chouchane, Ilaria Marinoni, Aurel Perren

Institution: University of Bern

Language: German

Topic: Neuroendocrine Tumor Research Advances

Subfield: Oncology

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: hybrid

Estimated APC paid: $3,390