Multiple endocrine neoplasia type 2: towards a risk-based approach integrating molecular and biomarker results.

Journal

Current opinion in oncology

ISSN: 1531-703X

Titre abrégé: Curr Opin Oncol

Pays: United States

ID NLM: 9007265

Informations de publication

Date de publication:
01 Jan 2024

Historique:

pubmed: 17 11 2023

medline: 17 11 2023

entrez: 17 11 2023

Statut: ppublish

Résumé

Significant advances have transformed our understanding of the molecular biology and natural history of multiple endocrine neoplasia type 2 (MEN2). This progress enacted a paradigm shift with regard to routine neck dissection for medullary thyroid cancer and total adrenalectomy for pheochromoytoma. The purpose of this review is to summarize key molecular and clinical data underpinning the current risk-based approach to MEN2 that integrates molecular and biomarker results. Early identification and biochemical monitoring of rearranged during transfection ( RET ) carriers yield important lead time. Within these ' windows of opportunity ', total thyroidectomy alone, avoiding incremental morbidity from node dissection; ' tissue-sparing ' subtotal adrenalectomy, balancing risks of steroid dependency with pheochromocytoma recurrence in adrenal remnants; and parathyroidectomy of enlarged glands only, weighing risks of postoperative hypoparathyroidism against hyperactive parathyroid glands left behind, are adequate therapies. All that is needed to determine a RET carriers' risk of medullary thyroid cancer, pheochromocytoma and/or primary hyperparathyroidism in the molecular era is patient age, underlying RET mutation, and biomarker levels. As broader testing begins to penetrate healthcare, the needle on population genomic screening and education needs to be moved forward to complete the transition from symptom-based to preventive healthcare.

Identifiants

DOI: 10.1097/CCO.0000000000001009 PMID: 37975407

pubmed: 37975407

doi: 10.1097/CCO.0000000000001009

pii: 00001622-990000000-00137

doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

Pagination

1-12

Informations de copyright

Références

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