Sporadic Medullary Thyroid Carcinoma: Towards a Precision Medicine.
RET gene
calcitonin
highly selective RET inhibitors
immunotherapy
medullary thyroid cancer
tyrosine kinase inhibitors
Journal
Frontiers in endocrinology
ISSN: 1664-2392
Titre abrégé: Front Endocrinol (Lausanne)
Pays: Switzerland
ID NLM: 101555782
Informations de publication
Date de publication:
2022
2022
Historique:
received:
28
01
2022
accepted:
04
03
2022
entrez:
15
4
2022
pubmed:
16
4
2022
medline:
19
4
2022
Statut:
epublish
Résumé
Medullary thyroid carcinoma (MTC) is a neuroendocrine malignant tumor originating from parafollicular C-cells producing calcitonin. Most of cases (75%) are sporadic while the remaining (25%) are hereditary. In these latter cases medullary thyroid carcinoma can be associated (multiple endocrine neoplasia type IIA and IIB) or not (familial medullary thyroid carcinoma), with other endocrine diseases such as pheochromocytoma and/or hyperparathyroidism. RET gene point mutation is the main molecular alteration involved in MTC tumorigenesis, both in sporadic and in hereditary cases. Total thyroidectomy with prophylactic/therapeutic central compartment lymph nodes dissection is the initial treatment of choice. Further treatments are needed according to tumor burden and rate of progression. Surgical treatments and local therapies are advocated in the case of single or few local or distant metastasis and slow rate of progression. Conversely, systemic treatments should be initiated in cases with large metastatic and rapidly progressive disease. In this review, we discuss the details of systemic treatments in advanced and metastatic sporadic MTC, focusing on multikinase inhibitors, both those already used in clinical practice and under investigation, and on emerging treatments such as highly selective RET inhibitors and radionuclide therapy.
Identifiants
pubmed: 35422765
doi: 10.3389/fendo.2022.864253
pmc: PMC9004483
doi:
Substances chimiques
Proto-Oncogene Proteins c-ret
EC 2.7.10.1
Types de publication
Journal Article
Review
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
864253Informations de copyright
Copyright © 2022 Matrone, Gambale, Prete and Elisei.
Déclaration de conflit d'intérêts
RE is consultant for Eisai, Loxo, Ipsen and Lilly, AM is consultant for Lilly, but the content of this paper was not influenced by this activity. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
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