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01-12-2013 | Case Report

The Diagnosis and Surgical Treatment of Pancreatoblastoma in Adults: A Case Series and Review of the Literature

Authors: Bulent Salman, Gabriel Brat, Yoo-Seok Yoon, Ralph H. Hruban, Aatur D. Singhi, Elliot K. Fishman, Joseph M. Herman, Christopher L. Wolfgang

Published in: Journal of Gastrointestinal Surgery | Issue 12/2013

Abstract

Introduction

Pancreatoblastoma is an extremely rare pancreatic neoplasm in adults. The aim of this study is to report our experience with adult pancreatoblastoma as well as review the cases reported in the literature in order to provide guidelines for the management of patients with this rare neoplasm.

Methods

We have encountered three cases of pancreatoblastoma in adults at our institution in addition to the 30 cases reported to date in literature.

Results

The median age of pancreatoblastoma in adults is 37 years (range, 18–78 years); men and women are similarly affected (male/female = 16/17). The behavior of pancreatoblastoma is clearly that of a malignant neoplasm, with local invasion, recurrence, and metastasis. Among the adult reported cases, at diagnosis or operation, metastasis and/or local invasion was found in 14 of 31 adult patients (46 %) (2 patients had no data) The survival was significantly higher in patients with resected tumor (resection only and resection + adjuvant chemo/radiotherapy) when compared to unresected patients (palliative chemo/radiotherapy and no treatment), (p = 0.008, HR = 0.20).

Conclusion

When disease is localized, the treatment of choice is a complete surgical resection. The role of adjuvant chemotherapy or radiotherapy is still unclear based on the very small number of patients treated.

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Title: The Diagnosis and Surgical Treatment of Pancreatoblastoma in Adults: A Case Series and Review of the Literature
Authors: Bulent Salman
Gabriel Brat
Yoo-Seok Yoon
Ralph H. Hruban
Aatur D. Singhi
Elliot K. Fishman
Joseph M. Herman
Christopher L. Wolfgang
Publication date: 01-12-2013
Publisher: Springer US
Published in: Journal of Gastrointestinal Surgery / Issue 12/2013
Print ISSN: 1091-255X
Electronic ISSN: 1873-4626
DOI: https://doi.org/10.1007/s11605-013-2294-2

Keynote webinar | Spotlight on medication adherence

Springer Medicine

The Diagnosis and Surgical Treatment of Pancreatoblastoma in Adults: A Case Series and Review of the Literature

Abstract

Introduction

Methods

Results

Conclusion

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Introduction

Methods

Results

Conclusion

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