Advertisement

 

 

Patient quality of life and prognosis in multiple endocrine neoplasia type 2.

Patient quality of life and prognosis in multiple endocrine neoplasia type 2.
Author Information (click to view)

Grey J, Winter K,


Grey J, Winter K, (click to view)

Grey J, Winter K,

Advertisement
Share on FacebookTweet about this on TwitterShare on LinkedIn

Endocrine-related cancer 2017 10 2425(2) T69-T77 doi 10.1530/ERC-17-0335

Abstract

Multiple endocrine neoplasia type 2 (MEN2) refers to the autosomal-dominant neuroendocrine tumour syndromes, MEN type 2A (MEN2A) and MEN type 2B (MEN2B). They are typified by the development of medullary thyroid cancer (MTC), phaeochromocytoma and parathyroid hyperplasia in MEN2A and MTC, phaeochromocytomas, ganglioneuromatosis and skeletal abnormalities in MEN2B. The aggressiveness of MTC is variable according to genotype, and although it is still the major cause of mortality in both conditions, prognosis has improved dramatically in those diagnosed and treated at a young age thanks to predictive genetic testing. Nevertheless, metastatic MTC, ganglioneuromatosis and a variety of other negative clinical and psychosocial impacts on quality of life and/or prognosis in MEN2 persist. In the absence, at the time of writing, of any large-scale research into quality of life specifically in MEN2, this review includes data from patient surveys and anonymised patient anecdotes from the records of the Association for Multiple Endocrine Neoplasia Disorders (AMEND), for whom the authors work. We recommend that these patients are cared for only in centres of expertise able to provide expert diagnosis, treatment and continuity of care, including psychological and transition support. Only in this way can the clinical advances of the last two and half decades be built upon further to ensure that the care of these complex, lifelong patients can be considered truly holistic.

Submit a Comment

Your email address will not be published. Required fields are marked *

twelve − 11 =

[ HIDE/SHOW ]