Uncommon Pancreatic Neoplasms

Transcription

1 Updates in Surgery

2 Paolo Pederzoli Claudio Bassi Editors Uncommon Pancreatic Neoplasms In collaboration with Massimo Falconi, Roberto Salvia and Giovanni Butturini Foreword by Gianluigi Melotti 123

3 Editors Paolo Pederzoli Department of Surgery and Oncology General Surgery Unit, Pancreas Center G.B. Rossi University Hospital Verona, Italy Claudio Bassi Department of Surgery and Oncology General Surgery Unit, Pancreas Center G.B. Rossi University Hospital Verona, Italy In collaboration with: Massimo Falconi, Roberto Salvia and Giovanni Butturini The publication and the distribution of this volume have been supported by the Italian Society of Surgery ISSN ISBN ISBN (ebook) DOI / Springer Milan Dordrecht Heidelberg London New York Library of Congress Control Number: Springer-Verlag Italia 2013 This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. Exempted from this legal reservation are brief excerpts in connection with reviews or scholarly analysis or material supplied specifically for the purpose of being entered and executed on a computer system, for exclusive use by the purchaser of the work. Duplication of this publication or parts thereof is permitted only under the provisions of the Copyright Law of the Publisher s location, in its current version, and permission for use must always be obtained from Springer. Permissions for use may be obtained through RightsLink at the Copyright Clearance Center. Violations are liable to prosecution under the respective Copyright Law. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. While the advice and information in this book are believed to be true and accurate at the date of publication, neither the authors nor the editors nor the publisher can accept any legal responsibility for any errors or omissions that may be made. The publisher makes no warranty, express or implied, with respect to the material contained herein Cover design: Ikona S.r.l., Milan, Italy Typesetting: Graphostudio, Milan, Italy Printing and binding: Arti Grafiche Nidasio S.r.l., Assago, Italy Printed in Italy Springer-Verlag Italia S.r.l. Via Decembrio 28 I Milan Springer is a part of Springer Science+Business Media (

4 Foreword It gives me great pleasure to introduce this comprehensive and exceptionally informative book on uncommon pancreatic neoplasms, including cystic, endocrine, and unusual solid tumors. Although individually they are deemed uncommon, viewed together these neoplasms are of very high clinical interest and importance. Furthermore, advances in imaging have made it possible to detect small lesions in the pancreas in asymptomatic patients, and the neoplasms discussed in this volume can frequently be treated by surgery alone or by a multimodal approach with curative intent. In bringing together Italian experts in pathology, imaging, surgery, and medical oncology to write this book, the editors, Professors Paolo Pederzoli and Claudio Bassi, have aimed to provide an integrated coverage that will acquaint clinicians and surgeons more closely with the described pathologies, reflecting their complexity and highlighting the state of the art in diagnosis and treatment, both surgical and medical. Without doubt they have succeeded in this goal! All aspects, from clinical presentation, genetics, and pathology through to diagnostic imaging, treatment, and follow-up, are considered with the degree of care expected by the discerning reader. It is my belief that the wide range of specialists involved in the care of patients with the described neoplasms will benefit enormously from this book, which provides all the tools needed for modern pathological classification and clinical management of these patients. Optimal surgical treatment is certainly based upon such a thorough multidisciplinary approach. My congratulations to Paolo and Claudio for this fine achievement! Rome, September 2012 Gianluigi Melotti President, Italian Society of Surgery v

5 Preface It is with a true sense of delight that I offer readers this book on rare pancreatic neoplasms. When the director of the Italian Society of Surgery (SIC) and my good friend Dr. Melotti invited me to choose a topic related to pancreatology as the subject of a book, I decided to focus on rare pancreatic neoplasms rather than on pancreatic ductal adenocarcinoma. The term "rare," as referred to pancreatic neoplasms, has recently become a matter of discussion, since the widespread use of more sophisticated imaging technologies has resulted in the detection of an increasing number of asymptomatic lesions in the pancreas. More frequently, these lesions are cystic in nature; only a few of them are neoplasms harboring malignant potential. Given that most of these conditions are scarcely known from a biological standpoint, their clinical management is challenging and highly debated. This book is divided into three parts, covering: cystic pancreatic neoplasms, neuroendocrine pancreatic neoplasms, and uncommon pancreatic solid neoplasms. Each section has been coordinated by an experienced pancreatic surgeon from our Surgical Department who is recognized for his expertise in the respective area. This experience has been broadened through collaborations with specialists from numerous other fields, including gastroenterologists, pathologists, oncologists, radiologists, and molecular biologists. Not surprisingly, the integration of these perspectives has been very successful, since multidisciplinarity is one of the main strengths of the Verona pancreatic group. The primary goal of this book is to offer readers a thorough, useful, readable, and understandable manual that presents an overview of rare pancreatic neoplasms and emphasizes the need for their multidisciplinary management. It is designed for rapid consultation whether by students, trainees, or experts in the field. I would like to thank Prof. Claudio Bassi in particular for joining me with great enthusiasm in the supervision of this book's preparation. I also thank the coordinators of each section, Prof. Massimo Falconi, Prof. Roberto Salvia, and Dr. Giovanni Butturini, for their excellent work. vii

6 viii Preface Finally, my appreciation is directed at all the coauthors involved in this project, for their helpfulness and remarkable dedication to the creation of an accessible, unique and up-to-date manual. I believe this collective effort will long serve as a valuable resource for readers interested in broadening their knowledge of some of the most fascinating and challenging topics in modern pancreatology. Verona, September 2012 Paolo Pederzoli

7 Preface It was during the early 1970s that Dr. Pederzoli, recently graduated from the prestigious University of Padua, decided to follow Professor Adamo Dagradi to the newly founded University of Verona. According to local legend, Paolo Pederzoli made this move since he had heard that in the city of Romeo and Juliet he would be able to focus on the pancreas. The same legend reports that the young pupil had to first ask his mentor: Ok, so where is the pancreas?. Many years have passed since that day, and time has flowed like the water of the Adige River, where it makes a U turn in the city of Verona, as if it did not want to leave its beauty (Berto Barbarani, Verona s most famous poet). That same Paolo Pederzoli who has never left his adopted city, has converted his devotion to the pancreas into a stunning professional career, recognized at the highest levels not only in Italy but also abroad. The secret of this success is contained in the following pages, which reveal a thorough and exhaustive knowledge of pancreatic diseases, including discussions of uncommon lesions rarely seen outside high-volume centers, and the logic underlying the adoption of a multidisciplinary approach to their diagnosis and management. This book presents the most recent findings on cystic, neuroendocrine, and other uncommon pancreatic neoplasms, with particular emphasis on their surgical pathology. A decision-making algorithm for each entity is provided in detail, while the above-mentioned multidisciplinary approach encompasses issues related to a variety of specialties, such as gastroenterology, oncology, endocrinology, and molecular biology. Clearly, this manual offers specialists in different areas of pancreatology a unique guide to uncommon pancreatic diseases. But are these diseases really uncommon? Certainly, a high-volume tertiary care center, such as the one directed for decades by Professor Pederzoli, cannot be considered representative of the outside reality. On the other hand, it is surprising how often a radiologist from a low-volume hospital must deal with small and asymptomatic pancreatic cysts. Our knowledge of these cysts has advanced to the stage that they can be regarded as extraordinary in vivo models whose developing pattern can be clinically monitored. ix

8 x Preface It is not only the morphologic features distinguished using sophisticated imaging techniques that have improved our understanding of pancreatic diseases. Tremendous advances also have been made in elucidating both the carcinogenic pathways that result in cystic neoplasms such as intraductal papillary mucinous neoplasms and the complex biology of neuroendocrine tumors. Collectively, a deeper understanding of these uncommon diseases, some of which are precursor lesions of ductal adenocarcinoma, will equip us with crucial models that can be applied to unravel the biology of their more frequently occurring malignant counterparts. Professor Pederzoli concludes his academic career by having involved all of his closest collaborators and colleagues in the creation of this valuable work: Thank you Paolo for your enthusiasm and for your passionate dedication to the challenging task of assembling this book. Your leadership, intense determination, and deep commitment to this wonderful profession that you have chosen will continue to inspire us in our own careers. Verona, September 2012 Claudio Bassi

9 Contents Part I Cystic Pancreatic Neoplasms Roberto Salvia 1 Classification Giuseppe Zamboni 2 Serous Cystic Neoplasms Giuseppe Malleo, Giuseppe Zamboni, Marina Paini, Giovanni Marchegiani and Riccardo Manfredi 3 Mucinous Cystic Neoplasms Giovanni Marchegiani, Riccardo Manfredi, Giuseppe Malleo, Isabella Frigerio and Giuseppe Zamboni 4 Solid-pseudopapillary, Acinar, and Other Cystic Neoplasms Marina Paini, Giuseppe Zamboni, Riccardo Manfredi, Salvatore Paiella and Giuseppe Malleo 5 Intraductal Papillary Mucinous Neoplasms Isabella Frigerio, Giuseppe Zamboni, Riccardo Manfredi, Antonio Pea, Silvia Pennacchio, Eugene Lim and Roberto Salvia 6 The Role of the Oncologist in the Diagnosis and Management of Malignant Cystic Neoplasms Alessandra Auriemma, Davide Melisi and Giampaolo Tortora xi

10 xii Contents Part II Neuroendocrine Pancreatic Neoplasms Massimo Falconi 7 Epidemiology and Clinical Presentation Maria Vittoria Davì, Marco Toaiari and Giuseppe Francia 8 Pathology and Genetics Aldo Scarpa and Vincenzo Corbo 9 Imaging Roberto Pozzi Mucelli, Giovanni Foti and Luigi Romano 10 Surgical Therapy Rossella Bettini, Stefano Partelli, Stefano Crippa, Letizia Boninsegna and Massimo Falconi 11 Functional Imaging and Peptide Receptor Radionuclide Therapy Maria Chiara Ambrosetti, Duccio Volterrani, Federica Guidoccio, Lisa Bodei, Federica Orsini, Giuliano Mariani and Marco Ferdeghini 12 Targeted and Other Non-receptor-mediated Therapies Sara Cingarlini, Chiara Trentin, Elisabetta Grego and Giampaolo Tortora Part III Uncommon Pancreatic Solid Neoplasms Giovanni Butturini 13 Rare Variants of Ductal Adenocarcinoma of the Pancreas Paolo Regi, Marco Dal Molin, Federica Pedica, Paola Capelli, Mirko D Onofrio and Giovanni Butturini 14 Rare Primary Tumors of the Pancreas Marco Dal Molin, Paola Capelli, Mirko D Onofrio, Ivana Cataldo, Giovanni Marchegiani and Giovanni Butturini 15 Rare Secondary Tumors of the Pancreas Giovanni Butturini, Marco Inama, Marco Dal Molin, Mirko D Onofrio, Davide Melisi, Giampaolo Tortora, Federica Pedica and Paola Capelli 16 Primary Non-epithelial Tumors of the Pancreas Marco Dal Molin and Paola Capelli

11 Contents xiii 17 Tumor-like Lesions of the Pancreas Luca Frulloni, Antonio Amodio, Italo Vantini, Marco Dal Molin, Marco Inama, Mirko D Onofrio, Lisa Marcolini, Claudio Luchini, Giovanni Butturini and Paola Capelli Index

12 Contributors Maria Chiara Ambrosetti Department of Pathology and Diagnostics, G.B. Rossi University Antonio Amodio Department of Medicine, Pancreas Center, G.B. Rossi University Alessandra Auriemma Department of Medical Oncology, G.B. Rossi University Claudio Bassi Department of Surgery and Oncology, General Surgery Unit, Rossella Bettini Department of Surgery and Oncology, G.B. Rossi University and General Surgery Unit, Sacro Cuore Don Calabria Hospital, Negrar (VR), Italy Lisa Bodei Division of Nuclear Medicine, European Institute of Oncology, Milan, Italy Letizia Boninsegna Department of Surgery and Oncology, G.B. Rossi University and General Surgery Unit, Sacro Cuore Don Calabria Hospital, Negrar (VR), Italy Giovanni Butturini Department of Surgery and Oncology, General Surgery Unit, Paola Capelli Department of Pathology and Diagnostics, Pathology Unit, G.B. Rossi University Ivana Cataldo Department of Pathology and Diagnostics, Pathology Unit, G.B. Rossi University Sara Cingarlini Department of Medical Oncology, G.B. Rossi University xv

13 xvi Contributors Vincenzo Corbo Department of Pathology and Diagnostics, Pathology Unit, G.B. Rossi University Hospital and ARC-NET Research Centre, University of Verona, Verona, Italy Stefano Crippa Department of Surgery and Oncology, G.B. Rossi University and General Surgery Unit, Sacro Cuore Don Calabria Hospital, Negrar (VR), Italy Marco Dal Molin Department of Surgery and Oncology, General Surgery Unit, Maria Vittoria Davì Department of Internal Medicine, G.B. Rossi University Mirko D Onofrio Department of Pathology and Diagnostics, Radiology Unit, G.B. Rossi University Massimo Falconi Department of Surgery and Oncology, G.B. Rossi University and General Surgery Unit, Sacro Cuore Don Calabria Hospital, Negrar (VR), Italy Marco Federghini Department of Pathology and Diagnostics, Radiology Unit, G.B. Rossi University Giovanni Foti Radiology Unit, Sacro Cuore - Don Calabria Hospital, Negrar (VR), Italy Giuseppe Francia Department of Internal Medicine, G.B. Rossi University Isabella Frigerio Surgery Unit, Casa di cura Dr. P. Pederzoli, Peschiera del Garda (VR), Italy Luca Frulloni Department of Medicine, Pancreas Center, G.B. Rossi University Elisabetta Greco Department of Medical Oncology, G.B. Rossi University Federica Guidoccio Regional Center of Nuclear Medicine, University of Pisa Medical School, Pisa, Italy Marco Inama Department of Surgery and Oncology, General Surgery Unit, Eugene Lim National University of Singapore Claudio Luchini Department of Pathology and Diagnostics, Pathology Unit, G.B. Rossi University

14 Contributors xvii Giuseppe Malleo Department of Surgery and Oncology, General Surgery Unit, Riccardo Manfredi Department of Pathology and Diagnostics, Radiology Unit, G.B. Rossi University Giovanni Marchegiani Department of Surgery and Oncology, General Surgery Unit, Lisa Marcolini Department of Pathology and Diagnostics, Pathology Unit, G.B. Rossi University Giuliano Mariani Regional Center of Nuclear Medicine, University of Pisa Medical School, Pisa, Italy Davide Melisi Department of Medical Oncology, G.B. Rossi University Federica Orsini Regional Center of Nuclear Medicine, University of Pisa Medical School, Pisa, Italy Salvatore Paiella Department of Surgery and Oncology, General Surgery Unit, Marina Paini Department of Surgery and Oncology, General Surgery Unit, Stefano Partelli Department of Surgery and Oncology, G.B. Rossi University and General Surgery Unit, Sacro Cuore Don Calabria Hospital, Negrar (VR), Italy Antonio Pea Department of Surgery and Oncology, General Surgery Unit, Paolo Pederzoli Department of Surgery and Oncology, General Surgery Unit, Federica Pedica Department of Pathology and Diagnostics, Pathology Unit, G.B. Rossi University Silvia Pennacchio Department of Surgery and Oncology, General Surgery Unit, Roberto Pozzi Mucelli Department of Pathology and Diagnostics, Radiology Unit, G.B. Rossi University Paolo Regi Surgery Unit, Casa di cura Dr. P. Pederzoli, Peschiera del Garda (VR), Italy Luigi Romano Radiology Unit, Sacro Cuore - Don Calabria Hospital, Negrar (VR), Italy

15 xviii Contributors Roberto Salvia Department of Surgery and Oncology, General Surgery Unit, Aldo Scarpa Department of Pathology and Diagnostics, Pathology Unit, G.B. Rossi University Hospital and ARC-NET Research Centre, University of Verona, Verona, Italy Marco Toaiari Department of Internal Medicine, G.B. Rossi University Giampaolo Tortora Department of Medical Oncology, G.B. Rossi University Chiara Trentin Department of Medical Oncology, G.B. Rossi University Italo Vantini Department of Medicine, Pancreas Center, G.B. Rossi University Duccio Volterrani Regional Center of Nuclear Medicine, University of Pisa Medical School, Pisa, Italy Giuseppe Zamboni Department of Pathology and Diagnostics, G.B. Rossi University and Pathology Unit, Sacro Cuore - Don Calabria Hospital, Negrar (VR), Italy