Dataset Information

Endocrine paraneoplastic syndromes in patients with neuroendocrine neoplasms.

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 (?-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?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.

SUBMITTER: Daskalakis K

PROVIDER: S-EPMC6531606 | biostudies-literature | 2019 May

REPOSITORIES: biostudies-literature

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Publications

Endocrine paraneoplastic syndromes in patients with neuroendocrine neoplasms.

Daskalakis Kosmas K Chatzelis Eleftherios E Tsoli Marina M Papadopoulou-Marketou Nektaria N Dimitriadis Georgios K GK Tsolakis Apostolos V AV Kaltsas Gregory G

Endocrine 20181002 2

<h4>Objective</h4>Our aim was to assess the prevalence of endocrine paraneoplastic syndromes (EPNS) in neuroendocrine neoplasms (NENs) and estimate its impact on patient outcomes.<h4>Design</h4>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 H ...[more]

PMID: 30280284

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Dataset Information

Endocrine paraneoplastic syndromes in patients with neuroendocrine neoplasms.

Objective

Design

Methods

Results

Publications

Endocrine paraneoplastic syndromes in patients with neuroendocrine neoplasms.

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