Hereditary Neuroendocrine Tumor Syndromes
نویسندگان
چکیده
Neuroendocrine tumours (NETs) are rare and heterogeneous neoplasms with variable bio‐ logical behaviour. The estimated incidence of NETs is about 1-5 cases/100,000/year. The most recent data show a progressive increase of the incidence in the last years and a high increase of their prevalence and survival [1]. NETs can be sporadic or can arise in complex hereditary endocrine disorders such as Multiple Endocrine Neoplasias (MENs), Familial Paragangliomatosis (FPGLs), Neurofibromatosis type 1 (NF1), von Hippel-Lindau Disease (VHL), Tuberous Sclerosis (TSC) [1]. It has been estimated that hereditary NETs occurrence varies with site of origin of the tumour, ranging 5 to 30% of cases [1]. Due to the recent ad‐ vances in the knowledge of biology and genetics of NETs, these rates seems to be an under‐ estimation and novel mutations of well known oncogenes or tumour suppressor genes as well as new genes and molecular pathways responsible for unknown syndromes are expect‐ ed to be characterized.
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