A variety of tumors originating from intra- or extra-abdominal viscera and, more rarely, from the peritoneal membrane, spread or metastasize to the visceral and parietal peritoneum. The term peritoneal surface malignancy (PSM) encompasses all these forms and thus identifies a heterogeneous family of primary or metastatic tumors with epithelial or mesenchymal origin. The inclusion of various forms of primary and secondary PSM under a unique definition is justified by the substantial uniformity of their clinical evolution within the abdominal and pelvic cavity, leading to production of tumor implants and ascites until fatal obstruction occurs. Prognosis is poor, and palliative therapy has long represented the only treatment option. In the natural history of PSM, evolution can be slow and metastatic development late, so that many forms represent ideal targets for aggressive locoregional therapies.A\In the 1980s, Paul Sugarbaker theorized - following countless pharmacokinet-ic and pharmacodynamic studies - ab ut advantages of the association between maximal surgical cytoteduction [peritonectomy (PRT)[, aimed at removing all visible implants, and hyperthermic intraperitoneal chemotherapy (HIPEC), aimed at treating microscopic or millimetric residues. Since the 1990s, this concept has gradually gained acceptance and currently is the intervention of choice for pseudomyx-oma peritonei and mesothelioma, but it is also diffusely used to treat carcinomato-sis from colorectal, gastric, and ovarian cancer and peritoneal sarcomatosis. For the most common forms of PSM treated with PRT plus HIPEC, experiences available to date consistently show overall results better than or highly competitive with traditional treatment modalities. PSM forms that until two decades ago were considered untreatable surgically and for which progression was fatal within months of diagnosis, today, after appropriate patient selection, are routinely treated with PRT plus HIPEC, resulting in improved patient quality of life and long-term survival ra es. The combined procedure achieves acceptable postoperative morbidity and mortality rates in relation to its complexity and duration (median 10 h) similar to those of major abdominal and pelvic surgery.A\However, the procedure has limited application considering the high overall incidence of various forms of PSM and is not exempt from criticism. The limited diffusion of PRT plus HIPEC treatment is related to the long learning curve; availability of relevant human, technical, and economic resources; and skepticism toward its effectiveness, particularly in reference to HIPEC, which is considered potentially risky during the postoperative course. Furthermore, the main criticisms concern the lack of prospective randomized phase III studies to define clearly the role of HIPEC, given that the validity of maximum cytoreduction is accepted worldwide. Indeed, to date, overall results of prospective trials for HIPEC are scarce and heavily criticized for the general treatment approach, lack of homogeneity of surgical techniques, and wide dispersion of enrolled cases. Therefore, results regarding overall significance of this procedure come mainly from multi-institutional studies, reviews, meta-analyses, and studies conducted in single centres with a high volume of PRT plus HIPEC activity. While taking into account the limitations inherent in such studies, the magnitude of experience gained to date reveals the overall trend of results. The great effort made by surgeons, oncologists, and specialized centers dedicated to treating PSM using PRT plus HIPEC has brought about the possibility of successfully treating aggressive locoregional tumors such as PSMs. It now remains for the inevitable upcoming prospective studies to confirm the promising results obtained thus far with this combined treatment modality and to determine the most appropriate ways to address treatment for PSM.A\The purpose of this monograph is to provide a summary of the knowledge base supporting the rationale of associating maximum cytoreduction with HIPEC, pathological assessment and diagnostic workup of patients with PSM, surgical and HIPEC techniques, and management results of the most common forms of PSM. In the world that revolves around PSM management, Italy plays a significant role, as demonstrated by case series treated by the various PSM centers in this country and the vast scientific contribution drawn from the literature and from acts of the major international conventions. Collaboration between many of the most important specialized Italian surgeons and treatment centers has helped provide an overall picture that illustrates the state of the art regarding PSM management. The topics discussed, and the opinions, experiences, and conclusions expressed by the various authors of these chapters, provide an in-depth summary of experiences pertaining to the most critical issues and outline goals to be achieved in the coming years through collective and coordinated efforts. Rome, September 2014, Angelo Di Giorgio, Enrico Pinto

Part I Background

1 Peritoneal Surface Malignancies 3

2 Epidemiology: Extent of the Problem 5

3 Mechanism of Intraperitoneal Spread of Free Cancer Cells 15

4 Pathology of Peritoneal Surface Malignancies 21

5 Classification of Intraperitoneal Spread 53

6 Diagnostic Imaging and Laparoscopy 69

Part II Treatment

7 Prevention and Management of Peritoneal Metastases from Gastrointestinal Cancer: A Short History of a Paradigm for Peritoneal Surface Malignancies 93

8 Rationale for Integrated Procedures: Cytoreduction and Hyperthermic Intraperitoneal Chemotherapy (HIPEC) Combined 107

9 Peritonectomy Techniques 129

10 Hyperthermic Intraperitoneal Chemotherapy (HIPEC) Techniques 155

11 The Role of Surgery 169

12 The Role of Systemic Chemotherapy 179

13 Patient Selection for Treatment 195

14 Morbidity and Mortality 207

15 Organizational Problems, Costs, and Data Collection 215

Part III Results of Integrated Treatment

16 Pseudomyxoma Peritonei 227

17 Peritoneal Mesothelioma 243

18 Peritoneal Carcinomatosis from Gastric Cancer 255

19 Peritoneal Carcinornatosis from Colorectal Cancer 271

20 Peritoneal Carcinomatosis from Ovarian Cancer 295

21 Other Primary Peritoneal Surface Malignancies 329

22 Other Secondary Peritoneal Surface Malignancies 339

23 Palliative Treatments 349

Part IV Perspectives

24 Main Topics of Discussion and New Trends 363

25 New Trials 375

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