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Aggressive behaviour of solid-pseudopapillary tumor of the pancreas in adults: a case report and review of the literature.

Authors
  • Sperti, Cosimo
  • Berselli, Mattia
  • Pasquali, Claudio
  • Pastorelli, Davide
  • Pedrazzoli, Sergio
Type
Published Article
Journal
World Journal of Gastroenterology
Publisher
Baishideng Publishing Group Co
Publication Date
Feb 14, 2008
Volume
14
Issue
6
Pages
960–965
Identifiers
PMID: 18240360
Source
Medline
License
Unknown

Abstract

Solid-pseudopapillary tumor (SPT) is a rare neoplasm of the pancreas that usually occurs in young females. It is generally considered a low-grade malignant tumor that can remain asymptomatic for several years. The occurrence of infiltrating varieties of SPT is around 10%-15%. Between 1986 and 2006, 282 cystic tumors of the pancreas were observed. Among them a SPT was diagnosed in 8 patients (2.8%) with only one infiltrating variety. This was diagnosed in a 49-year-old female 13 years after the sonographic evidence of a small pancreatic cystic lesion interpreted as a pseudocyst. The tumor invaded a long segment of the portal-mesenteric vein confluence, and was removed with a total pancreatectomy, resection of the portal vein and reconstruction with the internal jugular vein. Histological examination confirmed the R-0 resection of the primary SPT, although a vascular invasion was demonstrated. The postoperative course was uneventful, but 32 mo after surgery the patient experienced diffuse liver metastases. Chemotherapy with different drugs was started. The patient is alive and symptom-free, with stable disease, 75 mo after surgery. Twenty-five patients with invasion of the portal vein and/or of mesenteric vessels were retrieved from the literature, 16 recent patients with tumor relapse after potentially curative resection were also retrieved. The best treatment remains a radical resection whenever possible, even in locally advanced or metastatic disease. The role of chemotherapy, and/or radiotherapy, is still to be defined.

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