ECE2015 Guided Posters Endocrine tumours and neoplasia – NETS (9 abstracts)
Clinicopathologic features, treatments, and survival of patients with ectopic Cushing's syndrome from neuroendocrine tumours: data from an Italian multicentre study
Maria Vittoria Davi 1 , Elisa Cosaro 1 , Serena Piacentini 2 , Giuseppe Reimondo 3 , Nora Albiger 4 , Giorgio Arnaldi 5 , Antongiulio Faggiano 6 , Giovanna Mantovani 7 , Nicola Fazio 8 , Franco Grimaldi 9 , Massimo Mannelli 10 , Francesca Pecori Giraldi 11 , Chiara Martini 12 , Diego Ferone 13 , Davide Campana 14 , Carla Scaroni 4 , Massimo Terzolo 3 , Laura DeMarinis 2 & Giuseppe Francia 1
1University of Verona, Verona, Italy; 2Catholic University of Sacred Heart, Rome, Italy; 3University of Turin, Turin, Italy; 4Endocrinology, University of Padua, Padua, Italy; 5Polytechnic University of Marche Region, Ancona, Italy; 6Federico II University of Naples, Naples, Italy; 7Endocrinology, University of Milan, Milan, Italy; 8European Oncologic Institute, Milan, Italy; 9S. Maria della Misericordia Hospital, Udine, Italy; 10University of Florence, Florence, Italy; 11Istituto Auxologico Italiano, University of Milan, Milan, Italy; 12Clinica Medica III, University of Padua, Padua, Italy; 13University of Genoa, Genoa, Italy; 14University of Bologna, Bologna, Italy.
Introduction: Available literature on series of patients affected by ectopic Cushing’s syndrome (ECS) deriving from neuroendocrine tumours (NETs) is relatively scarce. This is the first Italian multicentre study regarding clinicopathologic features, modalities of treatment, and survival of patients with NETs and ECS.
Patients and methods: Retrospective analysis of data from patients with ECS from NETs collected in 14 centres between 1986 and 2014, obtained by a specific questionnaire.
Results: 96 patients, 58.3% females, mean (S.D.) age at diagnosis 50.4±15 years. Clinical presentation included hypertension (87%), diabetes mellitus (67%), proximal myopathy (67.7%), weight loss (28.1%), skin fragility (53%), hypercoagulopathy (28%), osteoporosis (47%), and psychiatric disease (32.3%). Regarding prevalence of NETs, 42.7% were bronchial carcinoids, 3.1% small-cell lung carcinoma (SCLC), 13.5% pancreatic (p), 8.3% thymic, 4.2% phaeochromocytoma, 5.2% others, and 23% occult. Tumour diameter of p-NETs was larger than that of bronchial carcinoids (47.3±34 mm vs 28±21.4 mm, P 0.035). Distant metastases were more prevalent in p-NETs, SCLC and thymic than in bronchial carcinoids and occult (77.7, 66.7, 50% vs 24.4, 13.6% respectively). Among 45 NETs with available Ki67, 46.7% were G1, 37.8% G2 and 15.6% G3. Immunostaining for ACTH was positive in 85.4%. Surgery was performed in 54,1%, steroidogenesis inhibitors in 77.1%, somatostatin analogues in 53.1%, cabergoline in 9.4%, mifepristone in 1%, everolimus in 6.2%, sunitinib in 1%, chemotherapy in 23%, PRRT in 10.4%, mono/bilateral adrenalectomy in 29%, and interventional treatments of liver metastases in 5.2%. Mean survival was 78 months (range 1–276), 24% died due to NET.
Conclusions: Bronchial carcinoids are the main NET associated with ECS, whereas 23% of cases are still occult. p-NETs are larger than bronchial carcinoids and more aggressive. Hystopathology, grading and distant metastases are the main prognostic factors. A multimodal treatment, including surgery of NET whenever possible, and adrenalectomy to definitively solve hypercortisolism thus reducing the risk of complications, can prolong survival.
Volume 37
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Endocrine Abstracts
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