Neuroendocrine neoplasms can occur as part of inherited disorders, usually in the form of well-differentiated, slow-growing tumors (NET). The main predisposing syndromes include: multiple endocrine neoplasias type 1 (MEN1), associated with a large spectrum of gastroenteropancreatic and thoracic NETs, and type 4 (MEN4), associated with a wide tumour spectrum similar to that of MEN1; von Hippel-Lindau syndrome (VHL), tuberous sclerosis (TSC), and neurofibromatosis 1 (NF-1), associated with pancreatic NETs. In the present review, we propose a reappraisal of the genetic basis and clinical features of gastroenteropancreatic and thoracic NETs in the setting of inherited syndromes with a special focus on molecularly targeted therapies for these lesions.
Neuroendocrine neoplasms in the context of inherited tumor syndromes: a reappraisal focused on targeted therapies / Ruggeri, R.M., Benevento, E., De Cicco, F., Fazzalari, B., Guadagno, E., Hasballa, I., Tarsitano, M.G., Isidori, A.M., Colao, A., Faggiano, A.. - In: JOURNAL OF ENDOCRINOLOGICAL INVESTIGATION. - ISSN 1720-8386. - 46:(2023), pp. 213-234. [10.1007/s40618-022-01905-4]
Neuroendocrine neoplasms in the context of inherited tumor syndromes: a reappraisal focused on targeted therapies
Benevento, E;De Cicco, F;Guadagno, E;Tarsitano, M G;Colao, A;Faggiano, A
2023
Abstract
Neuroendocrine neoplasms can occur as part of inherited disorders, usually in the form of well-differentiated, slow-growing tumors (NET). The main predisposing syndromes include: multiple endocrine neoplasias type 1 (MEN1), associated with a large spectrum of gastroenteropancreatic and thoracic NETs, and type 4 (MEN4), associated with a wide tumour spectrum similar to that of MEN1; von Hippel-Lindau syndrome (VHL), tuberous sclerosis (TSC), and neurofibromatosis 1 (NF-1), associated with pancreatic NETs. In the present review, we propose a reappraisal of the genetic basis and clinical features of gastroenteropancreatic and thoracic NETs in the setting of inherited syndromes with a special focus on molecularly targeted therapies for these lesions.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
