Endocrine paraneoplastic syndromes in patients with neuroendocrine neoplasms
| العنوان: | Endocrine paraneoplastic syndromes in patients with neuroendocrine neoplasms |
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| المؤلفون: | Daskalakis, Kosmas, Chatzelis, Eleftherios, Tsoli, Marina, Papadopoulou, Nektaria, Dimitriadis, Georgios K., Tsolakis, Apostolos V, Kaltsas, Gregory |
| المصدر: | Endocrine. 64(2):384-392 |
| مصطلحات موضوعية: | Paraneoplastic syndrome, Neuroendocrine tumours, Ectopic Cushings syndrome, Hypercalcitonaemia, PTHrP secretion |
| الوصف: | ObjectiveOur aim was to assess the prevalence of endocrine paraneoplastic syndromes (EPNS) in neuroendocrine neoplasms (NENs) and estimate its impact on patient outcomes.DesignThis is a retrospective analysis of 834 patients with NENs (611 gastrointestinal, 166 thoracic, 57 of unknown and various other primary origin). We included 719 consecutive NEN patients treated at EKPA-Laiko Hospital, Athens, Greece and 115 patients with lung carcinoid (LC) treated at Uppsala University Hospital, Uppsala, Sweden. EPNS diagnosis was based on standard criteria.MethodsTwenty-one patients with EPNS were detected: 16 with ectopic Cushings syndrome (ECS), one with hypercalcaemia due to parathyroid hormone-related protein (PTHrP) secretion, three with hypercalcitonaemia and one patient with dual secretion of calcitonin and beta-human chorionic gonadotropin (-HCG). All tumours were well-differentiated; 10 patients had Stage IV disease at diagnosis.ResultsThe prevalence of EPNS in the Greek cohort was 1.9%, whereas that of ECS among LC patients in both centres was 6.7%. Median overall survival (OS) for patients with EPNS was 160.7 months (95%CI, 86-235.4) and median event-free survival (EFS) was 25.9 months (95%CI, 0-57.2). Patients presenting with EPNS prior to NEN diagnosis had longer EFS compared to patients with synchronous or metachronous EPNS (log-rank P=0.013). Patients with ECS of extra-thoracic origin demonstrated shorter OS and EFS compared to patients with ECS of lung or thymic origin (log-rank P=0.001 and Pamp;lt;0.001, respectively). LC patients with and without ECS were comparable in 5-year and 10-year OS rates (66.7% and 33.3% versus 89.8% and 60.2%, respectively; 95%CI [189.6-300.4 months], log-rank P=0.94) and in median EFS, 67 versus 183 months, 95%CI [50.5-207.5], log-rank P=0.12).ConclusionEPNS are relatively rare in patients with NENs and mainly concern well-differentiated tumours of the foregut. Among patients with EPNS, LC-related ECS may not adversely affect patient outcomes when diagnosed prior to NEN and effectively been treated. |
| وصف الملف: | electronic |
| URL الوصول: | https://urn.kb.se/resolve?urn=urn:nbn:se:liu:diva-158354 https://liu.diva-portal.org/smash/get/diva2:1333839/FULLTEXT01.pdf |
| قاعدة البيانات: | SwePub |
Find this article in full text from Springer Verlag. | تدمد: | 1355008X 15590100 |
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| DOI: | 10.1007/s12020-018-1773-3 |