Hereditary syndromes associated with neuroendocrine tumors
نویسندگان
چکیده
منابع مشابه
Hereditary Neuroendocrine Tumor Syndromes
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 M...
متن کاملFamilial syndromes associated with neuroendocrine tumours
Neuroendocrine tumours may be associated with familial syndromes. At least eight inherited syndromes predisposing to endocrine neoplasia have been identified. Two of these are considered to be major factors predisposing to benign and malignant endocrine tumours, designated multiple endocrine neoplasia type 1 and type 2 (MEN1 and MEN2). Five other autosomal dominant diseases show more heterogene...
متن کامل[Associated gastroenteropancreatic neuroendocrine tumours to familiar syndromes].
Aproximately 5-10% of neuroendocrine tumours (NETs) of the gastroenteropancreatic system (GEP) have an hereditary background. The known hereditary syndromes include: multiple endocrine neoplasia type 1 (MEN 1), von Hippel Lindau disease (VHL), neurofibromatosis type 1 (NF 1) and tuberous sclerosis complex (TSC). This review discusses for each of these syndromes the: genes involved and specifics...
متن کاملHereditary paraganglioma-pheochromocytoma syndromes associated with SDHD and RET mutations.
BACKGROUND Hereditary paraganglioma-pheochromocytoma syndromes (PGL/PCC) are rare tumors arising from neuroendocrine cells. METHODS AND RESULTS The proband, a 59-year-old white man and his 42-year-old elder son had a medical history of bilateral carotid body PGL and both presented for treatment of abdominal PGLs. His 36-year-old daughter had excision of recurrent malignant carotid body PGL an...
متن کاملFactors Associated with Survival of Veterans with Gastrointestinal Neuroendocrine Tumors
Background. Gastrointestinal (GI) neuroendocrine tumor (NET) incidence has been increasing; however, GI NET within the national Veterans Affairs (VA) health system has not been described. Methods. We used the VA Central Cancer Registry to identify the cohort of patients diagnosed with GI NET in 1995-2009. Cox regression models were constructed to explore factors associated with survival. Result...
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ژورنال
عنوان ژورنال: Current Opinion in Endocrine and Metabolic Research
سال: 2021
ISSN: 2451-9650
DOI: 10.1016/j.coemr.2021.03.018