About the book
Neuroendocrine tumors (NETs) are rare neoplasms, they represent a heterogeneous group of malignant tumors that arise from the neuroendocrine system and can involve the entire body. NETs are responsible for about 0.5% of all cancers, and their incidence and prevalence have been increasing in the last three decades. These neoplasms are mostly non-functioning and are incidentally diagnosed, but the functioning ones are diagnosed with some clinical symptoms related to secreted bioactive amines. Neuroendocrine tumors include pheochromocytoma (PCC) and paraganglioma (PGL) with an incidence of about 0.6 cases per 100,000 person-years. In 2017, the WHO classification established that the pheochromocytoma is an adrenal tumor, and paraganglioma is an extra-adrenal tumor. Extraadrenal location may derive from sympathetic ganglia in the thorax, abdomen, and pelvis and parasympathetic paraganglia in the head and neck site. PCC and PGL present the involvement of more than 20 genes that are responsible for a hereditary background in up to 40%. Although progress has been made in the study of the pathogenetic mechanism, its full understanding is not achieved as well as the identification of specific targets of treatment.
This book will aim to provide an update on these rare neoplasms with an inevitable look at future treatment prospects.