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<p>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.</p><p>Compared to sporadic forms, hereditary NENs usually manifest at a&#xa0;younger age. Characteristic features include multifocal tumor development within a&#xa0;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&#xa0;guide for the mandatory molecular genetic germline analysis.</p><p>This overview explains the clinical and histological signs that may indicate a&#xa0;familial background.</p>

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Erbliche neuroendokrine Neoplasien

  • 期刊论文

  • 发表日期：

    2026年5月28日

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  • Aziz Chouchane,
  • Ilaria Marinoni,
  • Aurel Perren

摘要

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.