Natural history, treatment, and long-term follow up of patients with multiple endocrine neoplasia type 2B: an international, multicentre, retrospective study
The result's identifiers
Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00023761%3A_____%2F19%3AN0000008" target="_blank" >RIV/00023761:_____/19:N0000008 - isvavai.cz</a>
Alternative codes found
RIV/00216208:11130/19:10394266 RIV/00064203:_____/19:10394266
Result on the web
<a href="https://iris.uniroma1.it/retrieve/handle/11573/1224060/987016/Castinetti_Natural%20history_2019.pdf" target="_blank" >https://iris.uniroma1.it/retrieve/handle/11573/1224060/987016/Castinetti_Natural%20history_2019.pdf</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.1016/S2213-8587(18)30336-X" target="_blank" >10.1016/S2213-8587(18)30336-X</a>
Alternative languages
Result language
angličtina
Original language name
Natural history, treatment, and long-term follow up of patients with multiple endocrine neoplasia type 2B: an international, multicentre, retrospective study
Original language description
Multiple endocrine neoplasia type 2B is a rare syndrome caused mainly by Met918Thr germline RET mutation, and characterised by medullary thyroid carcinoma, phaeochromocytoma, and extra-endocrine features. Data are scarce on the natural history of multiple endocrine neoplasia type 2B. We aimed to advance understanding of the phenotype and natural history of multiple endocrine neoplasia type 2B, to increase awareness and improve detection. This study was a retrospective, multicentre, international study in patients carrying the Met918Thr RET variant with no age restrictions. The study was done with registry data from 48 centres globally. Data from patients followed-up from 1970 to 2016 were retrieved from May 1, 2016, to May 31, 2018. Our primary objectives were to determine overall survival, and medullary thyroid carcinoma-specific survival based on whether the patient had undergone early thyroidectomy before the age of 1 year. We also assessed remission of medullary thyroid carcinoma, incidence and treatment of phaeochromocytoma, and the penetrance of extra-endocrine features.
Czech name
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Czech description
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Classification
Type
J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database
CEP classification
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OECD FORD branch
30202 - Endocrinology and metabolism (including diabetes, hormones)
Result continuities
Project
<a href="/en/project/NV16-32665A" target="_blank" >NV16-32665A: Thyroid cancer in children and adolescents and its molecular genetic background</a><br>
Continuities
P - Projekt vyzkumu a vyvoje financovany z verejnych zdroju (s odkazem do CEP)
Others
Publication year
2019
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů
Data specific for result type
Name of the periodical
LANCET DIABETES & ENDOCRINOLOGY
ISSN
2213-8587
e-ISSN
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Volume of the periodical
7
Issue of the periodical within the volume
3
Country of publishing house
US - UNITED STATES
Number of pages
8
Pages from-to
213-220
UT code for WoS article
000459191900021
EID of the result in the Scopus database
2-s2.0-85061627076