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Endocrine paraneoplastic syndromes in patients with neuroendocrine neoplasms
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Surgical Sciences, Endocrine Surgery. Univ Athens, Laiko Hosp, Dept Propauped Internal Med 1, Oncol Unit, Athens, Greece.
Univ Athens, Laiko Hosp, Dept Propauped Internal Med 1, Oncol Unit, Athens, Greece;251 Hellen Air Force & VA Gen Hosp, Athens, Greece.
Univ Athens, Laiko Hosp, Dept Propauped Internal Med 1, Oncol Unit, Athens, Greece.
Linkoping Univ, Div Endocrinol, Dept Med & Hlth Sci, Linkoping, Sweden.
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2019 (English)In: Endocrine (Basingstoke), ISSN 1355-008X, E-ISSN 1559-0100, Vol. 64, no 2, p. 384-392Article in journal (Refereed) Published
Abstract [en]

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 (-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.

Place, publisher, year, edition, pages
SPRINGER , 2019. Vol. 64, no 2, p. 384-392
Keywords [en]
Paraneoplastic syndrome, Neuroendocrine tumours, Ectopic Cushing's syndrome, Hypercalcitonaemia, PTHrP secretion
National Category
Endocrinology and Diabetes Cancer and Oncology
Identifiers
URN: urn:nbn:se:uu:diva-386165DOI: 10.1007/s12020-018-1773-3ISI: 000468840200021PubMedID: 30280284OAI: oai:DiVA.org:uu-386165DiVA, id: diva2:1327989
Note

De två första författarna delar förstaförfattarskapet.

Available from: 2019-06-20 Created: 2019-06-20 Last updated: 2019-06-20Bibliographically approved

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