From biology to clinical experience: evolution in the knowledge of neuroendocrine tumours
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REVIEW
From biology to clinical experience: evolution in the knowledge of neuroendocrine tumours Emilio Bajetta Æ Giuseppe Procopio Æ Sara Pusceddu Æ Filippo Pietrantonio Æ Massimo Milione Æ Marco Maccauro Æ Elena Verzoni Æ Valentina Guadalupi Æ Marco Platania
Received: 20 February 2009 / Accepted: 22 April 2009 / Published online: 14 May 2009 Ó Springer-Verlag 2009
Abstract Neuroendocrine tumours (NETs) represent a group of neoplasias characterized by significant histopathological and biological heterogeneity. Diagnosis of neuroendocrine tumours relies upon histological examination augmented by newer techniques, such as pet-dotatoc scan. Surgery represents the only curative therapeutic approach. In advanced unresectable disease, medical treatment is the best choice. Somatostatin analogues allow adequate control of the carcinoid syndrome with a low effect on tumour cell growth. Chemotherapy has a good activity only in poor prognosis patients. According to the clinical course of the disease, a multimodal approach could be evaluated in selected cases. New target therapy including multikinase inhibitors, monoclonal antibody and m-tor inhibitors is now under clinical investigation for the treatment of advanced NETs. Preliminary results demonstrated a good activity in terms of disease control rate and tumour shrinkage. Keywords Neuroendocrine tumours Angiogenesis Metastasis Chemotherapy Target therapy
E. Bajetta (&) G. Procopio S. Pusceddu F. Pietrantonio E. Verzoni V. Guadalupi M. Platania Medical Oncology Unit 2, Fondazione IRCCS Istituto Nazionale dei Tumori, Via G. Venezian 1, 20133 Milan, Italy e-mail: [email protected] M. Milione Pathology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy M. Maccauro Nuclear Medicine Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
Introduction On account of natural history, clinical evolution and tumour biology, neuroendocrine tumours (NETs) represent a very heterogeneous group of neoplasms. With the purpose of categorizing the different prognostic variables, such as site and size of primary tumour, histological grading, proliferating index and release of biologically active substances, several classification models have been worked out. Up to now, none of these classifications appear completely comprehensive from the prognostic view point, as only a correct evaluation of the different clinical– pathological signs is truly predictive of the natural history of the disease. Due to their common neuro-ectodermal embryological origin, which is from neural crest, and only rarely endodermal origin, NETs can be ubiquitous; however, most frequent occurrences are seen in the gastrointestinal tract (65%) and in the bronchial–pulmonary tract (25%) [1]. The incidence of NETs has been estimated in about 2/100,000 cases per year. The stratification of the incidence rates according to age has identified two peaks: the first one in the age ranging from 15 to 25 years, and the second between 65 and 75 years [2]. Stage of t
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