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Endocrine paraneoplastic syndromes in patients with neuroendocrine neoplasms
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Daskalakisa, Kosmas, Chatzelis, Eleftherios, Tsolib, Marina, Papadopoulou, Nectaria, Dimitriadis, Georgios K., Tsolakise, Apostolos V. and Kaltsas, Gregory (2019) Endocrine paraneoplastic syndromes in patients with neuroendocrine neoplasms. Endocrine, 64 . pp. 384-392. doi:10.1007/s12020-018-1773-3 ISSN 1355-008X.
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Official URL: https://doi.org/10.1007/s12020-018-1773-3
Abstract
Objective
Our aim was to assess the prevalence of endocrine paraneoplastic syndromes (EPNS) in neuroendocrine neoplasms (NENs) and estimate its impact on patient outcomes.
Design
This 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.
Methods
Twenty-one patients with EPNS were detected: 16 with ectopic Cushing’s 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 (β-HCG). All tumours were well-differentiated; 10 patients had Stage IV disease at diagnosis.
Results
The 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 P < 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).
Conclusion
EPNS 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.
Item Type: | Journal Article | ||||||||
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Subjects: | R Medicine > RC Internal medicine > RC0254 Neoplasms. Tumors. Oncology (including Cancer) | ||||||||
Divisions: | Faculty of Science, Engineering and Medicine > Medicine > Warwick Medical School > Biomedical Sciences Faculty of Science, Engineering and Medicine > Medicine > Warwick Medical School > Biomedical Sciences > Translational & Experimental Medicine > Metabolic and Vascular Health (- until July 2016) Faculty of Science, Engineering and Medicine > Medicine > Warwick Medical School > Biomedical Sciences > Translational & Experimental Medicine Faculty of Science, Engineering and Medicine > Medicine > Warwick Medical School |
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Library of Congress Subject Headings (LCSH): | Neuroendocrine tumors, Paraneoplastic syndromes | ||||||||
Journal or Publication Title: | Endocrine | ||||||||
Publisher: | Springer | ||||||||
ISSN: | 1355-008X | ||||||||
Official Date: | 15 May 2019 | ||||||||
Dates: |
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Volume: | 64 | ||||||||
Page Range: | pp. 384-392 | ||||||||
DOI: | 10.1007/s12020-018-1773-3 | ||||||||
Status: | Peer Reviewed | ||||||||
Publication Status: | Published | ||||||||
Access rights to Published version: | Open Access (Creative Commons) | ||||||||
Date of first compliant deposit: | 25 September 2018 | ||||||||
Date of first compliant Open Access: | 17 January 2019 | ||||||||
RIOXX Funder/Project Grant: |
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