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      Successful Treatment of Solid-Pseudopapillary Tumor of the Pancreas with Multiple Liver Metastases

      case-report

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          Abstract

          The solid-pseudopapillary tumor (SPT) is a very rare pancreatic neoplasm that predominantly affects young females. About 450 cases have been described in the world literature and approximately 20% of the reported patients were children. The occurrence of SPT with distant metastases in children is extremely rare with only two previously reported cases. We now report a 16-year-old Asian girl with a large SPT and synchronous multiple liver metastases who was successfully treated in a 2-step strategy, including initial pylorus-preserving partial duodenopancreatectomy, right hemicolectomy, resection and allografting of the portal vein and secondary resection of 12 liver metastases. The patient is disease free after a follow-up of 18 months after resection of the primary tumor, suggesting that an aggressive surgical treatment might also be justified for metastasized SPT.

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          Most cited references13

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          Pancreatic solid-cystic-papillary tumor: clinicopathologic features in eight patients from Hong Kong and review of the literature.

          Solid-cystic-papillary tumors (SCPTs) of the pancreas are rare. The clinicopathologic features and pathogenesis of these tumors have attracted a number of investigations, but the results remain unclear. We investigated the clinicopathologic data, immunohistochemical expression of the pan-endocrine markers, hormones, steroid receptors, and p53 overexpression in pancreatic SCPTs from eight Chinese patients (seven women, one man) collected over a 24-year period. They accounted for 2.5% of the primary pancreatic tumors. The tumors were seen in young women (mean age 27 years). They were large (mean size of resected tumor was 8.4 cm), benign, had solid and cystic areas, and were evenly distributed in the pancreas. The main differential diagnosis was pancreatic endocrine tumor. The tumors were negative for pan-endocrine markers, hormones, estrogen receptor, progesterone receptor, and p53. To date, 452 pancreatic SCPTs have been documented in the English literature. They occurred in patients of different ethnic groups, particularly in non-Caucasians. The tumors were frequently noted in young females. Uncommon cases of malignant pancreatic SCPTs were often found in older men and had indolent behavior. It was concluded that pancreatic SCPTs have distinct clinicopathologic characteristics. The present observations, together with a review of the literature suggests that overexpression of p53 or estrogen receptor is not important in the pathogenesis of pancreatic SCPTs.
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            Papillary cystic tumors of the pancreas. Assessment of their malignant potential.

            Although the biologic characteristics of papillary cystic tumors (PCT) generally indicate a good prognosis, a malignant form has been reported. Twenty-two examples of PCT were examined to assess their malignant potential by histologic, flow cytometric, and immunohistochemical studies. Three had hepatic, peritoneal, and/or lymph node metastases (metastasizing tumors [MT]); the other 19 PCT had no such metastatic features (nonmetastasizing tumors [NMT]). Venous invasion was detected in all three MT and two NMT. Compared with the 19 NMT, the MT had a higher nuclear grade and more prominent necrobiotic nests characterized by aggregates of cells with pyknotic nuclei and eosinophilic cytoplasm. In the flow cytometric analysis of cellular DNA content, one MT was aneuploid, and eight NMT were diploid. Immunohistochemically, there was no difference between the MT and NMT. These results support the assumption that venous invasion, nuclear grade, and prominent necrobiotic nests are useful as histologic parameters to detect the malignant potential of PCT.
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              Solid-pseudopapillary tumor of the pancreas: A surgical enigma?

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                Author and article information

                Journal
                PAN
                Pancreatology
                10.1159/issn.1424-3903
                Pancreatology
                S. Karger AG
                1424-3903
                1424-3911
                2005
                May 2005
                09 May 2005
                : 5
                : 2-3
                : 289-294
                Affiliations
                aDepartment of Surgery and Institute of Theoretical Surgery, bInstitute of Pathology, and cDepartment of Diagnostic Radiology, Philipps University of Marburg, Marburg, Germany
                Article
                85285 Pancreatology 2005;5:289–294
                10.1159/000085285
                15855828
                728b23c6-e15a-4bdc-aca5-9273fbc3ce95
                © 2005 S. Karger AG, Basel and IAP

                Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

                History
                : 29 March 2004
                : 16 September 2004
                Page count
                Figures: 3, References: 25, Pages: 6
                Categories
                Case Report

                Oncology & Radiotherapy,Gastroenterology & Hepatology,Surgery,Nutrition & Dietetics,Internal medicine
                Liver metastases,Solid-papillary cystic tumor,Surgery

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