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NCPD Article
Evolving Foundations

Hereditary Neuroendocrine Tumors: Providing Comprehensive Care for Individuals Who Have a Germline Pathogenic Variant Associated With Paragangliomas and Pheochromocytomas

Suzanne M. Mahon
CJON 2021, 25(2), 137-141 DOI: 10.1188/21.CJON.137-141

Both paragangliomas and pheochromocytomas can be associated with germline pathogenic variants. Although these neuroendocrine tumors are relatively rare, the identification of patients and families with germline risk enables the implementation of surveillance programs to decrease the morbidity and mortality associated with these tumors. Individuals with germline risk require lifelong screening, which is implemented as early as age 5 years. In addition to ensuring that surveillance protocols are implemented, nurses provide education about symptoms that require prompt evaluation.

AT A GLANCE

  • Paragangliomas and pheochromocytomas are neuroendocrine tumors that are often the result of a germline pathogenic variant.
  • Individuals with a germline pathogenic variant associated with paragangliomas and pheochromocytomas require extensive monitoring starting at a young age and continuing throughout their life.
  • Extensive education and support are provided by nurses so that patients understand the importance of surveillance and reporting of any symptoms suggestive of excessive catecholamine release.

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