Current Treatment of Retroperitoneal Sarcomas

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1 Updates in Surgery

2 Vittorio Quagliuolo Alessandro Gronchi Editors Current Treatment of Retroperitoneal Sarcomas A Joint Effort with the Italian Society of Surgical Oncology In collaboration with Ferdinando C.M. Cananzi and Marco Fiore Foreword by Marco Montorsi 123

3 Editors Vittorio Quagliuolo Surgical Oncology Unit Department of Surgery Humanitas Clinical and Research Center Rozzano, Milan, Italy Alessandro Gronchi Sarcoma Service Department of Surgery Fondazione IRCCS Istituto Nazionale dei Tumori Milan, Italy The publication and the distribution of this volume have been supported by the Italian Society of Surgery ISSN Updates in Surgery ISBN ISSN (electronic) ISBN (ebook) DOI / Library of Congress Control Number: Springer-Verlag Italia S.r.l 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. 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. The publisher, the authors and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, express or implied, with respect to the material contained herein or for any errors or omissions that may have been made. The publisher remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. Cover design: estudio Calamar S.L. External publishing product development: Scienzaperta, Novate Milanese (Milan), Italy Typesetting: Graphostudio, Milan, Italy Printing and binding: Mariani Tipolitografia, Lissone (MB), Italy This Springer imprint is published by Springer Nature The registered company is Springer-Verlag Italia S.r.l. The registered company address is Via Decembrio 28, I Milan

4 Foreword In 1994 the Italian Society of Surgery inaugurated the series of Biennial Reports aiming at addressing the most current and controversial topics in surgery every year. One of the first two reports dealt with retroperitoneal tumors and was edited by Prof. Davide D Amico. After more than twenty years the Society s Executive Board has felt the need to fine-tune the theme of retroperitoneal neoplasias focusing on the most frequent and better studied form, namely sarcomas. All aspects of this complex disease have considerably evolved in recent years, enriched by new notions in both histological and molecular characterization, as well as in imaging, diagnostic pathways and surgical treatment, increasingly integrated into a multidisciplinary approach which is now essential. The Editors are two of the most prestigious and competent Italian surgeons, with vast experience on the subject and working in dedicated Institutions with high volumes of oncological activity. The different aspects of this important disease have been assigned to the most scientifically and clinically active Italian groups to ensure up-to-date and high level cultural development. The work ends with an interesting chapter that describes the path towards international collaboration of this group of experts to guarantee ever greater integration and exchange of experiences. I am sure that the monograph of Vittorio Quagliuolo and Alessandro Gronchi will be one of the most appreciated and consulted by the whole surgical community. Milan, September 2018 Marco Montorsi President, Italian Society of Surgery v

5 Preface Retroperitoneal sarcomas (RPS) are rare diseases accounting for 0.15% of all malignant tumors in the adult. Their treatment represents an extraordinary challenge in particular for the surgeon. Although other modalities may play a role in the management of RPS, surgery remains the cornerstone of therapy. The last 10 years have seen a major change in the surgical approach, with a substantial impact on disease control and cure. RPS referral centers with dedicated specialists and multidisciplinary tumor boards have become critical to improve disease-specific outcomes as compared with community/general hospitals without this expertise. National and international collaboration has grown significantly and has clearly proven to be instrumental for conducting research and advancing the knowledge of this rare disease. In this monograph, we have made an effort to summarize the most recent advances in RPS management: from diagnosis to the management of particular histological subtypes, from neoadjuvant therapies to extended multivisceral resections, with an eye to molecular biology and future perspectives. Current state-of-the-art knowledge about the various aspects of this complex disease is discussed, covering epidemiology, pathology, genetics, molecular biology, immunology, diagnostics, medical oncology, radiotherapy, prognosis prediction and of course surgical management of the different histological subtypes. The need to adopt a multidisciplinary approach and to regionalize RPS care in few referral centers across the country is often emphasized and is going to be the next challenge to face in the coming years, in order to further improve the prognosis of these patients while awaiting the development of new effective therapies. We wish to thank the President and the Board of the Italian Society of Surgery for giving us the opportunity and the task of editing this monograph. vii

6 viii Preface Special thanks also to all the contributors to this volume for spending their time to present the many complex aspects of this challenging disease. A particular thanks to the Italian Society of Surgical Oncology for the active collaboration which has been critical for the preparation of this volume. Milan, September 2018 Vittorio Quagliuolo Alessandro Gronchi

7 Contents 1 History of Surgery in Retroperitoneal Sarcomas... 1 Vittorio Quagliuolo, Laura Ruspi, Ferdinando C.M. Cananzi, and Alessandro Gronchi 2 The Pathology of Retroperitoneal Sarcomas... 9 Marta Sbaraglia, Piergiuseppe Colombo, and Angelo Paolo Dei Tos 3 The Complex Nature of Soft Tissue Sarcomas, Including Retroperitoneal Sarcomas Fabio Grizzi, Elena Monica Borroni, Dorina Qehajaj, Sanja Stifter, Maurizio Chiriva-Internati, and Ferdinando C.M. Cananzi 4 Imaging of Retroperitoneal Sarcomas Luca Balzarini, Nicolò Gennaro, and Carlo Morosi 5 Current Principles of Surgical Management of Retroperitoneal Sarcomas Marco Fiore and Sergio Sandrucci 6 Managing Early and Late Postoperative Complications Stefano Radaelli and Sergio Valeri 7 Major Vascular Resection in Retroperitoneal Surgery Ferdinando C.M. Cananzi, Laura Ruspi, Jacopo Galvanin, and Vittorio Quagliuolo 8 Pattern and Management of Recurrent Retroperitoneal Liposarcoma Elisabetta Pennacchioli, Massimo Barberis, and Stefania Rizzo ix

8 x Contents 9 Management of Other Recurrent Retroperitoneal Sarcomas Marco Rastrelli, Saveria Tropea, and Carlo Riccardo Rossi 10 The Role of Radiation Therapy in the Treatment of Retroperitoneal Sarcomas Antonino De Paoli, Federico Navarria, Elisa Palazzari, Piera Navarria, and Claudia Sangalli 11 Medical Therapy in Retroperitoneal Sarcomas Giovanni Grignani, Roberta Sanfilippo, and Alexia F. Bertuzzi 12 Predicting the Risk of Recurrence in Retroperitoneal Sarcoma Dario Callegaro, Alessandro Gronchi, Andrea Napolitano, and Bruno Vincenzi 13 Multimodal Management of Metastatic Disease Alexia F. Bertuzzi, Umberto Cariboni, Matteo M. Cimino, and Guido Torzilli 14 Postoperative Surveillance in Retroperitoneal Sarcomas Alessandro Comandone, Antonella Boglione, and Teresa Mele 15 Towards a Global Collaboration: the Trans-Atlantic Retroperitoneal Sarcoma Working Group Alessandro Gronchi and Vittorio Quagliuolo All web addresses have been checked and were correct at time of printing.

9 Contributors Luca Balzarini Department of Radiology, Humanitas Clinical and Research Center, Rozzano Massimo Barberis Department of Pathology, European Institute of Oncology, Milan, Italy Alexia F. Bertuzzi Department of Medical Oncology and Hematology, Humanitas Clinical and Research Center, Rozzano, Milan, Italy Antonella Boglione Department of Oncology, Humanitas Gradenigo Hospital, Turin, Italy Elena Monica Borroni Department of Biotechnology and Translational Medicine, University of Milan, Italy Dario Callegaro Sarcoma Service, Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy Ferdinando C.M. Cananzi Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, Milan, and Surgical Oncology Unit, Department of Surgery, Humanitas Clinical and Research Center, Rozzano, Milan, Italy Umberto Cariboni Department of Thoracic Surgery, Humanitas Clinical and Research Center, Rozzano, Milan, Italy Maurizio Chiriva-Internati Department of Lymphoma and Myeloma and Department of Gastroenterology, Hepatology and Nutrition, Division of Internal Medicine, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA Matteo M. Cimino Department of Hepatobiliary and General Surgery, Humanitas Clinical and Research Center, Rozzano, Milan, Italy xi

10 xii Contributors Piergiuseppe Colombo Department of Pathology, Humanitas Clinical and Research Center, Rozzano, Milan, Italy Alessandro Comandone Department of Oncology, Humanitas Gradenigo Hospital, Turin, Italy Antonino De Paoli Radiation Oncology Department, IRCCS CRO-Aviano, National Cancer Institute, Aviano, Pordenone, Italy Angelo Paolo Dei Tos University of Padua School of Medicine, Padua, Italy, and Department of Pathology, Azienda ULSS2 Marca Trevigiana, Treviso, Italy Marco Fiore Sarcoma Service, Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy Jacopo Galvanin Surgical Oncology Unit, Department of Surgery, Humanitas Clinical and Research Center, Rozzano, Milan, Italy Nicolò Gennaro Training School in Radiology, Humanitas University, Pieve Emanuele, Milan, Italy Giovanni Grignani Department of Medical Oncology, Candiolo Cancer Institute - FPO IRCCS, Candiolo, Turin, Italy Fabio Grizzi Department of Immunology and Inflammation, Humanitas Clinical and Research Center, Rozzano, Milan, Italy Alessandro Gronchi Sarcoma Service, Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy Teresa Mele Department of Oncology, Humanitas Gradenigo Hospital, Turin, Italy Carlo Morosi Department of Radiology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy Andrea Napolitano Medical Oncology Unit, Campus Bio-Medico University Hospital, Rome, Italy Federico Navarria Radiation Oncology Department, IRCCS CRO-Aviano, National Cancer Institute, Aviano, Pordenone, Italy Piera Navarria Radiotherapy and Radiosurgery Department, Humanitas Clinical and Research Center, Rozzano, Milan, Italy Elisa Palazzari Radiation Oncology Department, IRCCS CRO-Aviano, National Cancer Institute, Aviano, Pordenone, Italy

11 Contributors xiii Elisabetta Pennacchioli Soft Tissue Sarcoma and Rare Tumors Surgical Division, European Institute of Oncology, Milan, Italy Dorina Qehajaj Department of Immunology and Inflammation, Humanitas Clinical and Research Center, Rozzano, Milan, Italy Vittorio Quagliuolo Surgical Oncology Unit, Department of Surgery, Humanitas Clinical and Research Center, Rozzano, Milan, Italy Stefano Radaelli Sarcoma Service, Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy Marco Rastrelli Surgical Oncology Unit, Veneto Institute of Oncology IOV - IRCCS, Padua, Italy Stefania Rizzo, Department of Radiology, European Institute of Oncology, Milan, Italy Carlo Riccardo Rossi Surgical Oncology Unit, Veneto Institute of Oncology IOV - IRCCS, and Department of Surgery, Oncology and Gastroenterology, University of Padua, Padua, Italy Laura Ruspi Surgical Oncology Unit, Department of Surgery, Humanitas Clinical and Research Center, Rozzano, Milan, Italy Sergio Sandrucci Sarcoma and Rare Visceral Cancers Unit, Città della Salute e della Scienza, University of Turin, Turin, Italy Roberta Sanfilippo Adult Mesenchymal Tumors and Rare Cancers Medical Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy Claudia Sangalli Radiation Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy Marta Sbaraglia Department of Pathology, Azienda ULSS2 Marca Trevigiana, Treviso, Italy Sanja Stifter Department of Pathology, School of Medicine, University of Rijeka, Croatia Guido Torzilli Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, Milan, and Department of Hepatobiliary and General Surgery, Humanitas Clinical and Research Center, Rozzano, Milan, Italy Saveria Tropea Surgical Oncology Unit, Veneto Institute of Oncology IOV - IRCCS, and Department of Surgery, Oncology and Gastroenterology, University of Padua, Padua, Italy

12 xiv Contributors Sergio Valeri General Surgery Unit, Campus Bio-Medico University Hospital, Rome, Italy Bruno Vincenzi Medical Oncology Unit, Campus Bio-Medico University Hospital, Rome, Italy

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