Updates in Surgery

Vittorio Quagliuolo • Alessandro Gronchi Editors

Current Treatment of Retroperitoneal SarcomasA Joint Effort with theItalian Society of Surgical Oncology

In collaboration withFerdinando C.M. Cananzi and Marco Fiore

Foreword byMarco Montorsi

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EditorsVittorio Quagliuolo Alessandro GronchiSurgical Oncology Unit Sarcoma ServiceDepartment of Surgery Department of SurgeryHumanitas Clinical and Research Fondazione IRCCS Istituto NazionaleCenter dei TumoriRozzano, Milan, Italy Milan, Italy

The publication and the distribution of this volume have been supported by the Italian Society of Surgery

ISSN 2280-9848 ISSN 2281-0854 (electronic)Updates in SurgeryISBN 978-88-470-3979-7 ISBN 978-88-470-3980-3 (eBook)

DOI 10.1007/978-88-470-3980-3

Library of Congress Control Number: 2018948738

© Springer-Verlag Italia S.r.l. 2019

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Foreword

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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 MontorsiPresident, Italian Society of Surgery

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.

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Preface

Prefaceviii

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 QuagliuoloAlessandro Gronchi

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 ....................................................................... 21 Fabio Grizzi, Elena Monica Borroni, Dorina Qehajaj, Sanja Stifter, Maurizio Chiriva-Internati, and Ferdinando C.M. Cananzi

4 Imaging of Retroperitoneal Sarcomas ................................................... 33 Luca Balzarini, Nicolò Gennaro, and Carlo Morosi

5 Current Principles of Surgical Management of Retroperitoneal Sarcomas ....................................................................... 49 Marco Fiore and Sergio Sandrucci

6 Managing Early and Late Postoperative Complications ..................... 61 Stefano Radaelli and Sergio Valeri

7 Major Vascular Resection in Retroperitoneal Surgery ........................ 77 Ferdinando C.M. Cananzi, Laura Ruspi, Jacopo Galvanin, and Vittorio Quagliuolo

8 Pattern and Management of Recurrent Retroperitoneal Liposarcoma ............................................................................................. 93 Elisabetta Pennacchioli, Massimo Barberis, and Stefania Rizzo

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Contents

9 Management of Other Recurrent Retroperitoneal Sarcomas ................................................................................................... 109 Marco Rastrelli, Saveria Tropea, and Carlo Riccardo Rossi

10 The Role of Radiation Therapy in the Treatment of Retroperitoneal Sarcomas ................................................................... 121 Antonino De Paoli, Federico Navarria, Elisa Palazzari, Piera Navarria, and Claudia Sangalli

11 Medical Therapy in Retroperitoneal Sarcomas .................................... 133 Giovanni Grignani, Roberta Sanfilippo, and Alexia F. Bertuzzi

12 Predicting the Risk of Recurrence in Retroperitoneal Sarcoma ..................................................................................................... 143 Dario Callegaro, Alessandro Gronchi, Andrea Napolitano, and Bruno Vincenzi

13 Multimodal Management of Metastatic Disease ................................... 155 Alexia F. Bertuzzi, Umberto Cariboni, Matteo M. Cimino, and Guido Torzilli

14 Postoperative Surveillance in Retroperitoneal Sarcomas ................................................................................................... 165 Alessandro Comandone, Antonella Boglione, and Teresa Mele

15 Towards a Global Collaboration: the Trans-Atlantic Retroperitoneal Sarcoma Working Group ............................................ 173 Alessandro Gronchi and Vittorio Quagliuolo

All web addresses have been checked and were correct at time of printing.

Contentsx

Contributors

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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

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

xiiiContributors

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

Contributorsxiv

Sergio Valeri General Surgery Unit, Campus Bio-Medico University Hospital, Rome, Italy

Bruno Vincenzi Medical Oncology Unit, Campus Bio-Medico University Hospital, Rome, Italy