The Diagnosis and Medical Management of Advanced Neuroendocrine Tumors
Gregory A. Kaltsas,
G. Michael Besser and
Ashley B. Grossman
Department of Endocrinology, St. Bartholomew’s Hospital, London EC1A 7BE, United Kingdom
Correspondence: Address all correspondence and requests for reprints to: Prof. A. B. Grossman, Endocrine Oncology, Department of Endocrinology, St. Bartholomew’s Hospital, London EC1A 7BE, United Kingdom. E-mail: a.b.grossman{at}qmul.ac.uk
Neuroendocrine tumors (NETs) constitute a heterogeneous groupof neoplasms that originate from endocrine glands such as thepituitary, the parathyroids, and the (neuroendocrine) adrenal,as well as endocrine islets within glandular tissue (thyroidor pancreatic) and cells dispersed between exocrine cells, suchas endocrine cells of the digestive (gastroenteropancreatic)and respiratory tracts. Conventionally, NETs may present witha wide variety of functional or nonfunctional endocrine syndromesand may be familial and have other associated tumors. Assessmentof specific or general tumor markers offers high sensitivityin establishing the diagnosis and can also have prognostic significance.Imaging modalities include endoscopic ultrasonography, computedtomography and magnetic resonance imaging, and particularly,scintigraphy with somatostatin analogs and metaiodobenzylguanidine.Successful treatment of disseminated NETs requires a multimodalapproach; radical tumor surgery may be curative but is rarelypossible. Well-differentiated and slow-growing gastroenteropancreatictumors should be treated with somatostatin analogs or -interferon,with chemotherapy being reserved for poorly differentiated andprogressive tumors. Therapy with radionuclides may be used fortumors exhibiting uptake to a diagnostic scan, either aftersurgery to eradicate microscopic residual disease or later ifconventional treatment or biotherapy fails. Maintenance of thequality of life should be a priority, particularly because patientswith disseminated disease may experience prolonged survival.
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