Outcome and prognosis after pancreatectomy in patients with solid pseudopapillary neoplasms

Background - Solid pseudopapillary neoplasms (SPN) are rare and represent approximately 4% of all cystic pancreatic tumors. The prognosis is excellent, although 10-15% of SPN patients show metastasis at the time of surgery or tumor recurrence during follow-up after pancreatectomy. Aim of the study w...

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Main Authors: Tjaden, Christin (Author) , Hassenpflug, Matthias (Author) , Hinz, Ulf (Author) , Klaiber, Ulla (Author) , Klauß, Miriam (Author) , Büchler, Markus W. (Author) , Hackert, Thilo (Author)
Format: Article (Journal)
Language:English
Published: 14 June 2019
In: Pancreatology
Year: 2019, Volume: 19, Issue: 5, Pages: 699-709
ISSN:1424-3911
DOI:10.1016/j.pan.2019.06.008
Online Access:Verlag, Volltext: https://doi.org/10.1016/j.pan.2019.06.008
Verlag: http://www.sciencedirect.com/science/article/pii/S142439031930609X
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Author Notes:Christine Tjaden, Matthias Hassenpflug, Ulf Hinz, Ulla Klaiber, Miriam Klauss, Markus W. Büchler, Thilo Hackert

MARC

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520 |a Background - Solid pseudopapillary neoplasms (SPN) are rare and represent approximately 4% of all cystic pancreatic tumors. The prognosis is excellent, although 10-15% of SPN patients show metastasis at the time of surgery or tumor recurrence during follow-up after pancreatectomy. Aim of the study was to analyze surgical management, risk factors for malignancy as well as long-term outcome and prognosis of this distinct tumor entity. - Method - All patients with pancreatic resection for SPN between 10/2001 and 07/2018 in the authors’ institution were identified from a prospective database. Clinicopathologic details, perioperative data and long-term follow-up results were retrospectively analyzed. - Results - Fifty-two patients were identified, 44 (85%) of them were female and the median age was 29 years (IQR 9-71). Seven (13%) patients showed a malignant behaviour of SPN with N1 (n=2) or M1 (n=1) disease at resection; 5 patients developed tumor recurrence, after a median of 21 months. During follow-up time (median 54 months) all patients were alive, the 5- and 10-year rates for disease-free survival were 89.0% and 81.6%, respectively. Significant risk factors for recurrence were age <18 years (p=0.0087) and parenchyma-preserving surgical approaches (p=0.0006). The postoperative long-term outcome showed ECOG=0-1 in all patients, with resection related exocrine insufficiency in 20 (41%) and diabetes mellitus in 2 (4%) patients. - Conclusions - Age<18 years is a significant risk factor for malignancy in SPN, and parenchyma preserving resections harbor a significant risk for tumor recurrence. As recurrence may occur late, a systematic life-long follow-up should be performed. 
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