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Erschienen in: World Journal of Surgery 6/2015

01.06.2015 | Original Scientific Report

CUP Syndrome in Neuroendocrine Neoplasia: Analysis of Risk Factors and Impact of Surgical Intervention

verfasst von: Nehara Begum, Ulrich Wellner, Christoph Thorns, Georg Brabant, Martin Hoffmann, Conny Georg Bürk, Hendrik Lehnert, Tobias Keck

Erschienen in: World Journal of Surgery | Ausgabe 6/2015

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Abstract

Background

Neuroendocrine neoplasia (NEN) with unknown primary site (NEN-CUP tumors) may have a poor prognosis. We evaluated the clinical presentation, therapy, outcome, and risk factors for adverse outcomes in patients who had these tumors.

Methods

In 243 patients who had NEN, a retrospective review was performed in 38 patients who had NEN-CUP tumors. The 38 patients who had NEN-CUP tumors were evaluated in three groups: group 1 (surgery; primary tumor detected; ten patients); group 2 (surgery; no primary tumor detected; ten patients); and group 3 (no surgery; 18 patients). Risk factors were evaluated with univariate and multivariate analyses.

Results

Most patients who had NEN-CUP tumors [32 patients (84 %)] had World Health Organization (WHO) performance score of 0 or 1, and most tumors [24 patients (63 %)] were well differentiated (WHO grade, G1 or G2; Ki-67 index, ≤20 %). Univariate analysis showed that greater survival was significantly associated with lower patient age, lower WHO performance score, lower WHO grade, lower number of metastatic sites, treatment with surgery, and no treatment with chemotherapy. Multivariate analysis showed that low WHO performance score (hazard ratio 7.63, 95 % confidence interval (CI) 2.63–22.19) and treatment with surgery (hazard ratio 0.10, CI 0.028–0.381) were significant independent predictors of improved survival.

Conclusions

In patients with NEN-CUP tumors, surgical treatment is an independent predictor of better survival. Therefore, surgical treatment may be indicated in patients with good general health status and well-differentiated NEN-CUP tumors.
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Metadaten
Titel
CUP Syndrome in Neuroendocrine Neoplasia: Analysis of Risk Factors and Impact of Surgical Intervention
verfasst von
Nehara Begum
Ulrich Wellner
Christoph Thorns
Georg Brabant
Martin Hoffmann
Conny Georg Bürk
Hendrik Lehnert
Tobias Keck
Publikationsdatum
01.06.2015
Verlag
Springer International Publishing
Erschienen in
World Journal of Surgery / Ausgabe 6/2015
Print ISSN: 0364-2313
Elektronische ISSN: 1432-2323
DOI
https://doi.org/10.1007/s00268-015-2963-2

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