title: Thymic Neuroendocrine Neoplasms: Biological Behaviour and Therapy.
abstract: Thymic neuroendocrine neoplasms are rare tumours, but their management can often be highly problematic. While previously assumed to be essentially variants of bronchopulmonary (lung) carcinoids, they are generally more aggressive and more difficult to treat. Some 25% are associated with multiple endocrine neoplasia-1, while a higher proportion are associated with the ectopic ACTH syndrome, and occasionally both. We discuss the classification of these tumours, their biology as far as is known, and their clinical, biochemical and imaging features. We also review possible management options and suggest stratagems to optimise their treatment, which even today is far from optimal.
authors.affiliation: Department of Gastrointestinal Oncology, Affiliated Hospital Cancer Center, Academy of Military Medical Sciences, Beijing, China.
authors.forename: Ru
authors.initials: R
authors.lastname: Jia
authors.forename: Petra
authors.initials: P
authors.lastname: Sulentic
authors.forename: Jian-Ming
authors.initials: JM
authors.lastname: Xu
authors.forename: Ashley B
authors.initials: AB
authors.lastname: Grossman
mesh_terms: D006801:Humans; D018358:Neuroendocrine Tumors / Q000145:classification / Q000175:diagnosis / Q000503:physiopathology* / Q000628:therapy*; D013953:Thymus Neoplasms / Q000145:classification / Q000175:diagnosis / Q000503:physiopathology* / Q000628:therapy*
keywords: Bronchopulmonary carcinoids; Carcinoid; Ectopic ACTH; Multiple endocrine neoplasia-1; Neuroendocrine tumour; Thymus