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Aims: The classification of abdominal mucinous neoplasia is a controversial area. In 2010, WHO published a classification which divides pseudomyxoma peritonei (PMP) into low and high grades. The aim of the authors was to correlate this classification with the prognosis and site of primary neoplasm., Methods: The authors reviewed 274 patients with PMP who had undergone surgery at a single institution and classified them according to WHO criteria. The findings were correlated with clinical information and survival data., Results: PMP was low grade in 78% of patients and high grade in 22%. The appendix accounted for 94% of lesions, and the most common primary tumour was a low grade appendiceal mucinous neoplasm. Colorectal primaries were more likely to be associated with high grade PMP. There was an excellent correlation between the grade of the PMP and the primary neoplasm; only two cases showed discordant morphology: both were high grade appendiceal adenocarcinomas that were associated with low grade PMP. Nodal metastases were more likely in high grade lesions, but there was no significant difference in the rate of parenchymal organ invasion between low grade and high grade. Low grade morphology was associated with significantly longer survival than high grade (overall 5-year survival of 63% for low grade and 23% for high grade)., Conclusions: Categorisation as either low grade or high grade by WHO criteria correlates with prognosis. The grade of the PMP is generally consistent with the grade of the primary neoplasm. Colorectal primaries are more likely to be associated with high grade PMP.

Background: Pseudomyxoma peritonei (PMP) is a low-grade malignancy characterized by mucinous tumor on the peritoneal surface. Treatment involves cytoreductive surgery (CRS) to remove all macroscopic tumor and perioperative intraperitoneal chemotherapy (PIC) to eliminate remaining microscopic disease., Patients and Methods: Between 1994 and 2009, 93 patients were treated at the Norwegian Radium Hospital with complete CRS and PIC. PIC was administered as early postoperative intraperitoneal chemotherapy (EPIC) using mitomycin C (MMC) and 5-fluoruracil (n = 48) and as hyperthermic intraperitoneal chemotherapy (HIPEC) using MMC (n = 45). Patients were classified into three histopathological subgroups: Disseminated peritoneal adenomucinosis (n = 57), peritoneal mucinous carcinomatosis (n = 21) and an intermediate group (n = 15). Tumor distribution by peritoneal cancer index (PCI) was PCI ≤ 10 (n = 31), PCI 11-20 (n = 29), PCI ≥ 21 (n = 33)., Results: Recurrence was diagnosed in 38 patients and 25 patients died during follow-up. Estimated 10-year overall survival (OS) was 69% and 10-year disease-free survival (DFS) was 47%. Mean OS was 154 months (95% CI 131-171) and median OS was not reached (follow-up median 85 months (3-207)). Low-grade malignant histology (p = 0.001) and female gender (p = 0.045) were associated with improved OS. Almost equal OS and DFS were observed between patients treated with EPIC and HIPEC., Conclusions: Patients treated for PMP with complete CRS and PIC achieved satisfactory long-term outcome. The most important prognostic factor was histopathological differentiation, but acceptable survival was observed even in patients with aggressive histology and extensive intraperitoneal tumor growth. Administration of EPIC and HIPEC was equally efficacious with respect to long-term outcome., (Copyright © 2012 Elsevier Ltd. All rights reserved.)

Background: The aim of this study was to compare debulking surgery and cytoreductive surgery (CRS) in patients with Pseudomyxoma peritonei (PMP) regarding efficacy and safety., Patients and Methods: Data were extracted from medical records and treatment outcomes were analyzed for all 152 patients with PMP who were scheduled for debulking surgery and intraperitoneal chemotherapy (IPC) or CRS and IPC at Uppsala University Hospital, Uppsala, Sweden, between September 1993 and December 2008., Results: One hundred and ten patients (73%) were treated with CRS and IPC and 40 (27%) with debulking surgery and IPC. In two patients (1%), surgery was defined as open and close. Patients with CRS and IPC had a 74% 5-year overall survival (OS) rate compared with 40% for those treated with debulking surgery (P < 0.001). Patients with no residual macroscopic tumour (R1 resection) had a better 5-year OS rate of 94% compared with 28% for patients with macroscopic residual tumour (R2) (P < 0.001). Grades II-IV adverse events were seen in 29% of debulked patients and in 47% of CRS/IPC patients (P = 0.053)., Conclusions: CRS and IPC seems more efficient than debulking surgery and IPC but with numerically higher morbidity. Therefore, if surgically possible, CRS should be the treatment of choice for PMP patients. However, debulking surgery may still be of benefit to selected patients for palliative purposes., (Copyright © 2012 Elsevier Ltd. All rights reserved.)

Aims and Background: Pseudomyxoma peritonei (PMP) is a rare clinical entity characterized by diffuse intraabdominal gelatinous collections with mucinous implants on the peritoneal surfaces and omentum. This condition should be considered a borderline malignancy with disease progression over time. Encouraging treatment results have been recently reported with the combination of cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC)., Methods: . From December 2003 to December 2010, 18 patients with PMP were referred to our institution. All patients underwent peritonectomy and CRS combined with HIPEC in accordance with Sugarbaker's procedure., Results: The mean Peritoneal Cancer Index score was 27.6 (range, 5-39). Twelve (67%) patients had disseminated peritoneal adenomucinosis and 6 (33%) peritoneal mucinous carcinomatosis. Optimal cytoreduction with no visible residual disease or residual disease ≤2.5 mm in diameter was achieved in all patients. The mean duration of the surgical procedure including HIPEC was 9 hours and 30 minutes (range, 5-13 hours); major morbidity occurred in 30% of patients and the mortality was 11%. The mean follow-up was 27 months (range, 1-72) and the 5-year overall survival 66%., Conclusions: In line with the existing literature, our experience suggests that patients with PMP could benefit from CRS + HIPEC in terms of survival and locoregional disease control.

Background: The study examines the role inflammatory and tumor markers as biomarkers to preoperatively predict outcome in patients with epithelial appendiceal neoplasm undergoing cytoreduction and intraperitoneal chemotherapy., Methods: Associations between baseline variables, tumor markers [CEA (carcinoembyronic antigen], CA125, CA199), inflammatory markers including neutrophils-lymphocyte ratio (NLR), platelet-lymphocyte ratio (PLR), and C-reactive protein (CRP) with progression-free survival (PFS) and overall survival (OS) were examined in patients undergoing surgical cytoreduction and intraperitoneal chemotherapy for epithelial appendiceal neoplasm., Results: A total of 174 patients with epithelial appendiceal neoplasm (low-grade pseudomyxoma, n = 117; appendiceal cancer, n = 57) underwent cytoreduction. On univariate analysis, all 3 inflammatory and tumor markers predicted for both PFS and OS, respectively; NLR ≤ 2.6 (P = 0.01, P = 0.002), PLR ≤ 166 (P = 0.006, P = 0.016), CRP ≤ 12.5 (P = 0.001, P = 0.008), CEA (P < 0.001, P = 0.001), CA125 (P = 0.004, P < 0.001), CA199 (P < 0.001, P < 0.001). On multivariate analysis, there were no independent predictors of OS. PFS was independently associated with the presence of lymph node metastasis (P = 0.02), CA199 > 37 (P = 0.003), and a CRP > 12.5 (P = 0.013). A higher peritoneal cancer index (PCI > 24) was associated with elevation in CEA > 12, CA125 > 39, CA199 > 37, PLR > 166 and CRP > 12. The tumor histologic subtype was associated with CA 199 levels., Conclusions: The results from this investigation suggest that preoperative inflammatory markers in blood and serologic tumor markers may predict outcomes and are associated with tumor biology in patients with epithelial appendiceal neoplasm undergoing cytoreduction and intraperitoneal chemotherapy treatment.

Purpose: Pseudomyxoma peritonei (PMP) originating from an appendiceal mucinous neoplasm remains a biologically heterogeneous disease. The purpose of our study was to evaluate outcome and long-term survival after cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) consolidated through an international registry study., Patients and Methods: A retrospective multi-institutional registry was established through collaborative efforts of participating units affiliated with the Peritoneal Surface Oncology Group International., Results: Two thousand two hundred ninety-eight patients from 16 specialized units underwent CRS for PMP. Treatment-related mortality was 2% and major operative complications occurred in 24% of patients. The median survival rate was 196 months (16.3 years) and the median progression-free survival rate was 98 months (8.2 years), with 10- and 15-year survival rates of 63% and 59%, respectively. Multivariate analysis identified prior chemotherapy treatment (P < .001), peritoneal mucinous carcinomatosis (PMCA) histopathologic subtype (P < .001), major postoperative complications (P = .008), high peritoneal cancer index (P = .013), debulking surgery (completeness of cytoreduction [CCR], 2 or 3; P < .001), and not using HIPEC (P = .030) as independent predictors for a poorer progression-free survival. Older age (P = .006), major postoperative complications (P < .001), debulking surgery (CCR 2 or 3; P < .001), prior chemotherapy treatment (P = .001), and PMCA histopathologic subtype (P < .001) were independent predictors of a poorer overall survival., Conclusion: The combined modality strategy for PMP may be performed safely with acceptable morbidity and mortality in a specialized unit setting with 63% of patients surviving beyond 10 years. Minimizing nondefinitive operative and systemic chemotherapy treatments before definitive cytoreduction may facilitate the feasibility and improve the outcome of this therapy to achieve long-term survival. Optimal cytoreduction achieves the best outcomes.

Aim: To assess the clinicopathologic features and its relationship with prognosis of pseudomyxoma peritonei (PMP) in Chinese patients., Methods: The clinicopathologic features and follow-up data of 92 patients with PMP were reviewed and retrospectively analyzed. The cases were categorized into three groups: disseminated peritoneal adenomucinosis (DPAM), peritoneal mucinous carcinomatosis (PMCA), and peritoneal mucinous carcinomatosis with intermediate or discordant features (PMCA-I/D). The log-rank test was used to analyze survival for each group and various clinicopathological parameters. Multivariate Cox proportional-hazard models were constructed to determine the important factors associated with survival., Results: The median age at diagnosis was 51.9 years (range: 22-76 years). The median follow up was 124 mo. The 3-, 5- and 10-year survival rates were 74.0%, 67.4% and 49.1%, respectively. There were 49 (53.2%) patients with DPAM, 26 (28.3%) with PMCA-I and 17 (18.5%) with PMCA. Patients with DPAM, PMCA-I/D and PMCA exhibited statistically significant difference in survival (P = 0.001). The 3 year survival for DPAM, PMCAI/D and PMCA was 97.0%, 80.0% and 67.0%, respectively; the 5 year survival was 80.0%, 67.0% and 50.0%, respectively; and the 10 year survival was 65.0%, 28.0% and 14.0%, respectively. Survival rate was significantly lowest in patients < 40 age years of age (P = 0.011). Appendiceal tumor and extra-ovarian parenchymal organ involvement were significantly related to overall survival. Patients with appendiceal mucinous adenocarcinoma (MACA) showed the significantly poorer prognosis (P = 0.011). Multivariate analysis showed that pathological classification, age, appendiceal tumor were significant related to overall survival., Conclusion: The clinical process "PMP" should be pathologically classified into DPAM, PMCA and PMCA-I/D. Pathological classification, age, appendiceal MACA are survival independent predictors in Chinese patients with PMP.

Background/aims: Pseudomyxoma peritonei syndrome (PMP) may be associated with slow "benign" or malignant process. However, the natural history of this disease is slow progression to death. Its treatment is variable and controversial. In this current study we have compared the patients with Pseudomyxoma peritonei syndrome with recurrences due to the cell entrapment hypothesis., Methodology: Data were derived from a database of patients with PMP treated at our hospitals from 2004 to 2009. All patients had undergone various surgical operations prior to referral to our institutions for definitive treatment. All patients had recurrences in special sites due to entrapment of malignant cells., Results: There are 6 patients, four men and two women. The initial clinical presentation of the disease was hernia in one, appendicitis in three and ovarian mass in one. The mean time from the initial operation to be definitive management was 23.5 months with an average of 1.83 operations per patient. After cytoreduction and hyperthermic intraperitoneal chemotherapy (HIPEC) the mean survival was 31 months with minimal recurrences and only 0.3 operations per patient., Conclusions: Our data suggest that the patients should be referred to a center with a peritoneal surface malignancy program after the PMP diagnosis as soon as possible. Incomplete debulking procedures and minimal invasive operations promote uncontrollable intraabdominal tumor growth due to tumor cell entrapment and the tendency of PMP to grow at wound sites.

Background: Most cytoreduction with hyperthermic intraperitoneal chemotherapy procedures are performed at academic tertiary referral centers with numerous surgical oncology faculty. The objective of this study was to review the postoperative morbidity and mortality data of our institution, a large community hospital., Methods: This was a retrospective cohort study of patients who underwent cytoreduction with hyperthermic intraperitoneal chemotherapy at a single institution. Two surgical oncologists performed all the procedures between May 2005 and June 2011., Results: We retrospectively analyzed 57 patients. The most common pathology being treated was pseudomyxoma peritonei (34 of 57; 59.6%), followed by colorectal cancer (9 of 57; 15.8%). Other types of cancer included peritoneal mesothelioma and gastric adenocarcinoma. The average surgery time was 6.9 hours. Approximately 51% of patients suffered grade 3 or 4 morbidity and there were no perioperative mortalities., Conclusions: Cytoreduction with hyperthermic intraperitoneal chemotherapy can be performed at our institution with comparable outcomes as academic referral centers., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Mucinous neoplasms of the appendix are rare tumors, some of them characterized by an enigmatic discrepancy between a benign morphologic appearance and an aggressive biologic potential, associated with a poor prognosis and high mortality. The clinical picture of pseudomyxoma peritonei is, with few exceptions, caused by mucinous appendiceal neoplasms and differs in many aspects from usual peritoneal carcinomatosis. The controversy regarding terminology, diagnostic criteria, classification and therapy of these tumors has lasted for decades. The revised edition of the World Health Organization Classification of Tumors of the Digestive System proposes a uniform reporting system for mucinous appendiceal neoplasms and the peritoneal disease associated with it, thereby creating a comparable basis for pathological diagnosis, clinical therapy and further scientific studies.

Pseudomyxoma peritonei (PMP) is primarily the result of a ruptured mucinous appendix neoplasm (MAN). Often MAN is lumped with but biologically distinct from intestinal appendiceal adenocarcinoma. Nodal and systemic dissemination are rare with the peritoneal cavity being the primary site of recurrence. Routine performance of right hemicolectomy (RHC) for PMP/MAN has been extensively debated without consensus. Our objective was to ascertain whether RHC has a survival advantage over appendectomy. We hypothesize if RHC is mandatory, then increased tumor recurrence and mortality should be observed in appendectomy only. Retrospective chart review was carried out in patients with tumors that met the Ronnett classification for PMP/MAN. Demographics, tumor grade, extent, recurrence, and progression were recorded. We report the rate of nodal involvement/recurrence in patients treated with RHC versus appendectomy as well as the rate of systemic and peritoneal recurrence and survival. Multivariate logistic regression was done to identify factors that impact survival. Of 120 patients, 48 had appendectomy and 72 had RHC. Seven per cent of patients undergoing RHC had positive lymph nodes and no nodal failures (0%) in patients undergoing appendectomy. Appendectomy versus RHC recurrence rates (21 vs. 28%, P = 0.12) and death resulting from disease (8 vs. 22%, P = 0.27) were similar. Logistic regression revealed that the type of surgery had no impact on recurrence and mortality, only optimal resection score and performance status. There was no difference in tumor recurrence or survival based on treatment by appendectomy or RHC. Performance status and complete cytoreduction are the only factors associated with survival. Lymph node involvement is rare and selective RHC is safe in PMP/MAN.

Purpose: It has been reported that complete cytoreduction using peritonectomy combined with intraperitoneal chemotherapy improves the prognosis of patients with pseudomyxoma peritonei (PMP); however, this treatment strategy remains controversial, especially at nonspecialized institutes, because of its high morbidity rate., Methods: We reviewed the clinical records of 15 consecutive patients with PMP, treated in nonspecialized hospitals and observed by one of us between 1999 and 2010. Cytoreductive surgery was done using peritonectomy procedures with intraperitoneal chemotherapy and was performed with curative intent, |in accordance with Sugarbaker., Results: All patients had mucinous tumors disseminated in the peritoneal cavity. Complete cytoreduction was achieved in 12 patients. Morbidity was 40% (6/15) and mortality was 0% (0/15). After a median follow-up period of 43 months, the 12 patients who underwent complete cytoreduction were disease-free with good quality of life, and 1 of the 3 patients who underwent incomplete cytoreduction was alive with disease., Conclusions: These findings suggest that peritonectomy with intraperitoneal chemotherapy for PMP can provide prognostic benefit, even at nonspecialized hospitals. Considering the treatment risk, it should ideally be performed at a referral center, or at least by an experienced surgeon.

Background: Cytoreductive surgery (CRS) combined with perioperative intraperitoneal chemotherapy (PIC) is a recognized management strategy for pseudomyxoma peritonei. We seek to evaluate the outcomes of patients treated upfront with CRS PIC compared to patients undergoing delayed CRS PIC as salvage or treatment for recurrences after initial debulking surgery., Methods: Retrospective analysis of patients with low-grade pseudomyxoma peritonei treated within our institution were stratified according to upfront versus delayed CRS PIC after intial debulking surgery. Survival was analyzed using the Kaplan-Meier method and compared using the log-rank test., Results: Of 83 patients treated, 35 patients (42%) underwent upfront and 48 patients (58%) underwent delayed CRS PIC. The peritoneal cancer index (P = 0.048), amount of blood transfusion intraoperatively (P = 0.003) and duration of operation (P = 0.007) was lesser in the upfront compared to delayed group. Upfront treatment confers 5-year recurrence-free survival benefit (77% vs 37%; P = 0.011) and 10-year overall survival benefit (67% vs 35%; P = 0.054) over delayed treatment., Conclusion: Upfront CRS PIC seems to confer beneficial perioperative outcomes and lower recurrence rates over delayed CRS PIC. Early referral to centralized treatment centers would seem to be a reasonable strategy to improve outcomes.

Background: Pseudomyxoma peritonei syndrome is a clinical entity characterized by mucinous ascites usually originating from a perforated mucinous appendiceal tumor. Currently optimal therapy is considered complete macroscopic tumor removal (complete cytoreduction) combined with heated intraperitoneal chemotherapy. When complete cytoreduction is not achievable, major tumor debulking is undertaken. The long-term follow-up of patients undergoing surgery for perforated appendiceal tumors in a national pseudomyxoma center is reported., Methods: Between March 1994 and July 2009, 456 patients with pseudomyxoma peritonei syndrome from perforated appendiceal tumors underwent surgery. The treatment strategy involved a combination of cytoreductve surgery aiming for macroscopic tumor removal, combined with hyperthermic intraperitoneal chemotherapy with mitomycin C. Where complete tumor removal was not possible, maximal tumor debulking was performed. Perioperative outcomes and predicted 5- and 10-year survival are reported., Results: Overall 15 of 456 (3%) tumors were not resectable at laparotomy. Of the 441 patients who had resection, 289 (66%) had complete cytoreduction and 152 (34%) had major tumor debulking. Postoperative in-hospital mortality was 1.6% and grade 3/4 morbidity was 7%. The Kaplan-Meier method predicted 5- and 10-year overall survival of 69% and 57%, respectively. Five- and 10-year predicted survival was 87% and 74% for the 289 patients who had complete cytoreduction compared with 34% and 23% for the 152 who had major tumor debulking., Conclusion: Complete tumor removal was achieved in 289 of 441 (66%) patients with peritoneal dissemination from perforated appendiceal tumors with good long-term survival and probable cure in more than two-thirds.

Pseudomyxoma peritonei (PMP) is a rare condition of mucinous ascites associated mainly with mucinous tumors of appendix or ovary. PMP often recurs after treatment and may eventually cause death by abdominal visceral dysfunction via compression with mucinous ascites. Although radical peritonectomy and hyperthermic intra-peritoneal chemotherapy are becoming popular globally, the optimal treatment of PMP has not been established in Japan. We conducted a retrospective multicenter study to clarify the optimal treatment and the prognostic factors of PMP. A total of 23 patients with PMP were analyzed in the Tohoku Gynecologic Cancer Unit (TGCU). Clinical and follow-up data were retrieved and a central pathology review was performed. The median follow-up period was 46 months. Eleven patients underwent complete resection. There were 7 deaths out of 13 recurrences/progressions in this period. All the recurrence/progression was confined to the abdomen. Unexpectedly, neither radical peritonectomy nor hyperthermic intra-peritoneal chemotherapy had been performed, indicating that both radical peritonectomy and hyperthermic intra-peritoneal chemotherapy are not yet popular in Japan. The medians of overall survival and disease-free period were 166 months and 30 months, respectively. Univariate and multivariate analyses revealed that the only prognostic factor was macroscopic residual tumor (P=0.022). Although chemotherapy was not a prognostic factor (P=0.16), those who received intra-peritoneal chemotherapy tended to have a better prognosis than those who received systemic or no chemotherapy (P=0.064). In conclusion, the macroscopic residual tumor is an important prognostic factor in Japanese patients with PMP.

The goal of this study is to evaluate the use of positron emission tomography (PET) in evaluation of patients with peritoneal dissemination of carcinoma of appendiceal origin (PDA). Thirty-three patients with PDA, who had preoperative PET or PET/CT imaging, were analyzed. Using operative, pathology, and PET +/- CT data, presence or absence of disease in each abdominal quadrant was noted and the use of 18fluoro-deoxy-glucose (FDG) PET for each quadrant was evaluated. The mean age was 52, and there were 17 males; 58 per cent had low-grade lesions. PET was positive in only 35 per cent of cases overall (30 and 41% sensitivity for low-grade and high-grade, respectively). PET without CT sensitivity for low-grade and high-grade lesions was 21 and 8 per cent, respectively. PET imaging has limited use for patients with PDA. We do not recommend the use of FDG-PET for patients with PDA from cancer of the appendix.

Background: Cytoreductive surgery (CRS) combined with perioperative intraperitoneal chemotherapy (PIC) has demonstrated improved survival in selected patients with pseudomyxoma peritonei (PMP). However, this aggressive treatment modality has been consistently associated with variable rates of perioperative mortality between 0% and 18% and morbidity between 30% and 70%. This study evaluates the clinical and treatment-related risk factors for perioperative morbidity and mortality in PMP patients who underwent CRS and PIC., Materials and Methods: A total of 145 consecutive CRS and PIC procedures for PMP performed between January 1996 and March 2009 were evaluated. The association of 12 clinical and 20 treatment-related risk factors with grades III and IV/V morbidity were assessed by univariable and multivariable analysis., Results: The mortality (grade V) rate was 3%. The morbidity rates of grades III and IV were 23% and 22%, respectively. Eight factors were associated with grade IV/V morbidity on univariable analysis: peritoneal cancer index >or=21 (P = .034), ASA score >or=3 (P = .003), operation duration >or=10 h (P < .001), left upper quadrant peritonectomy procedure (P = .037), colonic resection (P = .012), ostomy (P = .005), ileostomy (P = .012), and transfusion >or=6 units (p = 0.011). Multivariable analysis showed 2 significant risk factors for grade IV/V morbidity: ASA >or= 3 (P = .006) and an operation length >or=10 h (P < .001)., Conclusions: CRS and PIC has an acceptable rate of perioperative mortality and morbidity in selected patients with PMP. Patients with bulky disease who undergo a long operation are at a particularly high risk of a severe adverse event.

Introduction: Peritoneal carcinomatosis (PC) is one of the routes of dissemination of abdominal neoplasms and is generally considered a lethal disease, with a poor prognosis by conventional chemotherapeutic treatments. While systemic chemotherapy has little impact on the treatment of peritoneal disease, some centers have reported encouraging results with cytoreduction and hyperthermic intraperitoneal chemotherapy (HIPEC). This approach is based on surgical cytoreduction of the primary tumour, peritonectomy (stripping of implants on the peritoneal surface) and HIPEC. The rationale of this treatment, after macroscopic disease removal, is to obtain an elevated and persistent drug concentration in the peritoneal cavity, with limited systemic effects. Many studies have reported encouraging results on overall survival (OS) and the disease-free interval in patients affected by PC., Patients and Methods: From October 1997 to November 2008, 411 operations for PC were performed in our institution; in 232 cases, cytoreduction plus HIPEC was carried out. Out of 72 operations for colonic cancer: 40 cytoreductions plus HIPEC, 12 cytoreductions+ EPIC (early postoperative intraperitoneal chemotherapy) and 16 debulking or explorative laparoscopies/laparotomies were performed. For the present study, the 40 patients who had undergone cytoreduction plus HIPEC for PC of colorectal cancer (CRC) were considered., Results: The complication rate was 55% (22/40) and mortality rate 2.5% (1/40). The specific features of both groups were considered for the survival curves and complication rates, with special reference to the peritoneal carcinomatosis index (PCI; range 0, absence of disease to 39) and completeness of cytoreduction score (CCR; 0, no residual tumor, to CCR 3, residual nodules greater than 25 mm). In Group A, patients operated on prior to 2002, the median survival time was 16.7 months compared to 24.6 months for Group B, those operated on after 2002. The poor survival of Group A seemed to be related to higher PCI and CCR scores., Conclusion: Correct patient selection based on a maximum PCI of 16, associated with complete cytoreduction (CCR-0), produced encouraging results in our experience. To improve this encouraging survival outcome, it is very important to unify the surgical experience of expertise centres. Our results also suggest the need for an integrated approach to this condition to identify the correct aspect of the surgical domain and results that may be influencing the prognosis and the evolution of this patients.

Background: Two main treatment options are available for pseudomyxoma peritonei (PMP) and disseminated peritoneal adenomucinosis (DPAM)--incomplete cytoreductive (debulking) surgery and peritonectomy with intraperitoneal chemotherapy. Several studies have demonstrated improved survival with peritonectomy. This study analyses outcome following peritonectomy in patients undergoing a primary procedure compared to those who have had previous debulks., Methods: Between April 1997 and May 2008, 63 patients underwent peritonectomy for DPAM--38 had had previous debulk(s) and 25 underwent primary peritonectomy. Patients were followed-up at three- to six-monthly intervals postoperatively, with a mean follow-up time of 21 and 34 months, respectively., Results: Mean survival for patients undergoing primary peritonectomy was 109.8 months. Mean survival for patients with previous debulks was 49.2 months (p=0.027). Five-year survival was 95.5% in the primary peritonectomy group and 67.5% in the previous debulk group., Conclusion: Disease recurrence after primary peritonectomy for DPAM is significantly less frequent than after secondary peritonectomy.

The treatment of peritoneal carcinomatosis represents a challenge in the therapy for gastrointestinal cancer. A multimodal approach with complete surgical cytoreduction and hyperthermic intraperitoneal chemotherapy can improve the prognosis in selected patients. Complete surgical cytoreduction, consisting of parietal and visceral peritonectomy, is a sophisticated procedure, frequently requiring multivisceral resections and should only be performed by experienced visceral surgeons. In addition, hyperthermic intraperitoneal chemotherapy is of some complexity. Furthermore, regarding the learning curve for this procedure, combined treatment should only be performed in specialised centres. Under optimal conditions, the therapy can be carried out with reasonable morbidity and mortality rates. Patients with peritoneal carcinomatosis should be evaluated by an interdisciplinary team concerning this multimodal therapy option and, if applicable, they should be referred to therapy within the framework of clinical studies., ((c) Georg Thieme Verlag Stuttgart-New York.)

Peritoneal surface malignancy is the expression of a spectrum of disease involving the peritoneum primary or secondary to gastrointestinal and gynecological neoplasms. Even if intraperitoneal therapy has now been demonstrated in multiple randomized trials to improve the outcome of chemotherapy for patients with optimally debulked or small volume ovarian carcinoma, it is believed that peritoneal carcinomatosis is considered an advanced stage of disease; for this reason, it is treated with systemic chemotherapy and surgery plays only a palliative role (1). In the last twenty years, some centres have developed surgical treatment of peritoneal carcinomatosis that involves aggressive cytoreductive surgery associated with hyperthermic intraperitoneal chemotherapy. This treatment has improved and prolonged survival, despite the associated high morbidities and mortalities (3-14).

Background: Pseudomyxoma peritonei (PMP) is a rare peritoneal carcinomatosis, characterized by a slowly progressive disease process with a large amount of mucus containing occasional epithelial cells. PMP is histologically classified into disseminated peritoneal adenomucinosis (DPAM), peritoneal mucinous carcinomatosis (PMCA) and an intermediate or discordant feature group (ID). Recent studies have shown that most cases of PMP originate from ruptured appendiceal tumors with progressive dissemination in the peritoneal cavity of mucin-producing epithelial cells. Encouraging results in the treatment of PMP have been reported by surgical cytoreduction of the primitive cancer, peritonectomy (stripping of implants on the peritoneal surface) and intraperitoneal hyperthermic chemoperfusion (HIPEC). In recent trials, this combined approach has been proposed as the standard treatment for PMP., Patients and Methods: In this study, the results of twelve years single-institution experience on 60 consecutive patients affected by PMP, treated by cytoreductive surgery and the original semi-closed HIPEC technique are reported with special reference to overall survival (OS) and progression-free survival (PFS)., Results: The postoperative morbidity rate was 45% (27 patients); surgical morbidity was observed in 19 patients and medical complications in 9 cases. No postoperative deaths were observed. The survival data, 53 patients were analized (the last 7 were considered only for the complications rate, postoperative mortality and cancer features, not for OS or PFS because they were too recent for evaluation). At the final follow-up of the 53 patients, five and ten years OS were respectively 94% and 84.6% . DFS was 80% and 70% at five and ten years respectively. The follow-up data indicated that the survival probability may be good in patients with hystological type appendicular adenoma optimally cytoreduced (CCR-0). Interestingly if preoperative chemotherapy was performed represented a negative prognostic factor with statistically significant impact both on OS and DFS., Conclusion: As in other similar studies, cytoreductive surgery plus HIPEC, even when combined with an aggressive surgical procedure, is associated with an acceptable risk of postoperative complications and mortality. This combined treatment results in DFS and OS rates that are not described in the literature for surgery associated with systemic chemotherapy and, in our opinion, may be considered the gold standard treatment for this rare tumor.

Background: Cytoreductive surgery (CRS) and perioperative intraperitoneal chemotherapy (PIC) has been recognized as a treatment option for pseudomyxoma peritonei. This study reports the survival outcomes, clinicopathological prognostic factors, and a learning curve from a single institution's experience., Methods: Patients with pseudomyxoma peritonei underwent CRS and PIC, which was comprised of hyperthermic intraperitoneal chemotherapy (HIPEC) and/or early postoperative intraperitoneal chemotherapy (EPIC), according to a standardized treatment protocol in our institution. Clinicopathological factors were analyzed to determine their prognostic value for survival using univariate and multivariate analysis. Time period comparison was performed to study the effect of a learning curve., Results: A total of 106 patients (43 men and 63 women) were treated. The mortality rate was 3% and severe morbidity rate was 49%. The median follow-up was 23 (range, 0-140) months. The overall median survival was 104 months with a 5-year survival rate of 75%. The progression-free survival was 40 months with a 1-year progression-free survival rate of 71%. Factors influencing survival include histopathological type of tumor, use of both HIPEC and EPIC, peritoneal cancer index, completeness of cytoreduction, and severe morbidity. The results demonstrate a learning curve where patients with a higher peritoneal cancer index (PCI) were treated, reduced amount of blood products required, more patients undergoing HIPEC and the combined HIPEC and EPIC, more redo-procedures performed, and a longer progression-free survival., Conclusions: This report demonstrates long-term survival outcomes, acceptable perioperative outcomes, and a learning curve associated with the treatment of patients with pseudomyxoma peritonei.

Background: Pseudomyxoma peritonei is derived mostly from the rupture of an appendiceal cystic tumor and produces a large quantity of mucinous substance. Though its malignancy level is low, radical resection is difficult and its prognosis is poor. In some institutes in European countries and the United States, multidisciplinary treatment with chemo-hyperthermia at a relatively low temperature is performed following subtotal peritonectomy. We carried out high-temperature chemo-hyperthermic peritoneal perfusion following incomplete resections of mucinous tumors in six patients., Methods: After resection of the main tumor and macroscopic gross tumor resection of dissemination, heated perfusate containing anticancer agents was poured into the peritoneal cavity and stirred and pumped into a circulation between the abdomen and a reservoir. The temperature of the surface of the peritoneum was maintained at around 43 degrees C., Results: The intraperitoneal space was sufficiently heated without severe complications. The pathological diagnosis in four of the patients was peritoneal mucinous carcinomatosis, and in the other two patients, it was intermediate type between peritoneal mucinous carcinomatosis and disseminated peritoneal adenomucinosis. Two patients died, 15 and 26 months after the treatment. The other four patients have survived for 4, 24, 26, and 80 months now, and two of them, who have survived for 26 and 80 months, received a second treatment 18 and 32 months, respectively, after the initial treatment. These results seemed not to be inferior to those in the reports from institutes in Europe and the United States which performed subtotal peritonectomy and then used mild hyperthermia in chemo-hyperthermia., Conclusion: For treating pseudomyxoma peritonei, high-temperature chemo-hyperthermic peritoneal perfusion following incomplete tumor resection is effective even without peritonectomy.

Background: Peritoneal carcinomatosis has a typical natural history of bowel obstruction and death. Significant evidence suggests that cytoreduction with heated intraperitoneal chemotherapy (HIPEC) improves long-term survival for these tumors., Methods: A retrospective case series of patients who underwent initial HIPEC treatment was performed at 2 moderate-volume centers. Clinicopathologic data were reviewed and univariate analyses performed to determine predictors of periprocedural complications., Results: Twenty-eight patients underwent HIPEC procedures. The most common pathologies were colonic adenocarcinoma and pseudomyxoma peritonei. The median preoperative peritoneal cancer index was 9.5. Thirteen patients had 34 complications, with no postoperative deaths. Pleural effusion and wound infection were the most common complications. Preoperative performance status and the extent of disease were predictive of complications., Conclusions: Cytoreduction and HIPEC can be done at moderate-volume centers with morbidity and mortality rates comparable with published results from large-volume centers. Preoperative performance status and the extent of disease predict postoperative complications.

Aim: To investigate the most important aspects of hyperthermic intraperitoneal chemotherapy (HIPEC) that has been accepted as the standard treatment for pseudomyxoma peritonei (PMP), with special regard to morbidity, overall survival (OS) and disease free survival (DFS) over 10 years., Methods: Fifty-three patients affected by PMP underwent cytoreduction (CCR) and HIPEC with a "semi-closed" abdomen technique in our institution. The peritonectomy procedure and completeness of CCR were classified according to Sugarbaker criteria. Preoperative evaluation always included thoracic and abdominal CT scan to stage peritoneal disease and exclude distant metastases. Fifty-one patients in our series were treated with a protocol based on administration of cisplatinum 100 mg/m(2) plus mitomycin C 16 mg/m(2), at a temperature of 41.5 degrees centigrade for 60 min. Anastomoses were always performed at the end of HIPEC. The mean duration of surgery was 12 h including HIPEC. Continuous monitoring of hepatic and renal functions and hydroelectrolytic balance was performed in the postoperative period., Results: Twenty-four patients presented with postoperative complications: surgical morbidity was observed in 16 patients and 6 patients were re-operated. All complications were successfully treated and no postoperative deaths were observed. Risk factors for postoperative morbidity were considered to be gender, age, body surface, duration of surgery, Peritoneal Cancer Index (PCI) and tumor residual value (CC score). No statistically significant correlation was found during the multivariate analysis: only the CC score was statistically significant. The OS in our experience was 81.8%, with a DFS of 80% at 5 years and of 70% at 10 years., Conclusion: In our experience, even if HIPEC combined with cytoreductive surgery involves a high risk of morbidity, postoperative complications can be resolved favorably in most cases with correct patient selection and adequate postoperative care, thus minimizing mortality. The association of CCR and HIPEC can be considered as the standard treatment for PMP. The OS and DFS results confirm the validity of this combined approach for the treatment of this rare neoplasm. The impact of preoperative chemotherapy on OS, in our opinion, is due to a major aggressiveness of tumors in treated patients.

Background: Pseudomyxoma peritonei (PMP) is characterized by progressive intraperitoneal accumulation of mucous and mucinous implants, usually derived from a ruptured, possibly malignant mucinous neoplasm of the appendix. Treatment based on complete cytoreductive surgery (CCRS) with hyperthermic intraperitoneal chemotherapy (HIPEC) is gaining support. The aim of this study was to identify pre- and perioperative factors of prognostic value., Methods: A total of 105 patients (with no residual tumours exceeding 2 mm) were treated with CCRS plus HIPEC based on oxaliplatin. Clinical, radiological, pathological factors and blood markers were analysed to determine their prognostic value for survival., Results: Mortality (7.6 per cent) and morbidity (67.6 per cent) were significantly correlated with peritoneal index, pathological grade and blood CA19.9 level. The median follow-up was 48 months. Seven patients died after hospital discharge. Overall and disease-free 5-year survival rates were 80.0 and 68.5 per cent respectively. The Cox model identified only two significant factors impacting on disease-free survival: CA19.9 level and pathological grade., Conclusion: CCRS is the most effective treatment for PMP, and adding HIPEC prolongs long-term survival. Further strategies should focus on improving postoperative outcome in extended PMP.

Background: Surgical cytoreduction combined with hyperthermic intraperitoneal chemotherapy (HIPEC) has been recently advocated as the standard of care for pseudomyxoma peritonei (PMP). We reviewed our 10-year monoinstitutional case series to identify selection factors predicting postoperative outcome., Methods: One hundred and four patients with PMP were operated on with the aim of performing adequate cytoreduction (residual tumor nodules < or =2.5 mm) and closed-abdomen HIPEC with mytomicin-C and cisplatin. Previously, 26 patients had systemic chemotherapy. PMP was histologically classified into disseminated peritoneal adenomucinosis (DPAM), peritoneal mucinous carcinomatosis (PMCA), and intermediate/discordant group (ID). Immunohistochemical stains were performed for cytokeratin (CK)-7, CK-20, CDX-2, MUC-2, MUC-5AC, CD-44s. The significance of 22 potential clinical, pathological, and biological prognostic variables was assessed by multivariate analysis., Results: Adequate cytoreduction was performed in 89 patients, suboptimal cytoreduction in six, palliative surgery in nine. Operative mortality was 1%. Seventy-eight patients were diagnosed with DPAM, 26 with PMCA, and none with ID. Median follow-up was 37 months (range, 1-110) for the overall series. Five-year overall survival (OS) and progression-free survival (PFS) were 78.3% and 31.1%, respectively. At multivariate analysis, adequate cytoreduction, no previous systemic chemotherapy, and DPAM correlated to better OS and PFS, elevated serum CA19.9 correlated only to better PFS. In most cases, CK20, CDX-2, and MUC-2 were diffusely positive, while CK-7, MUC-5AC, and CD44s were variably expressed. CK20 expression correlated to prognosis at univariate analysis., Conclusions: Favorable outcome after comprehensive treatment can be expected in patients with DPAM, not treated with preoperative systemic chemotherapy and amenable to adequate cytoreduction. MUC-2, CK-20, and CD44s expression may be related to PMP unique biologic behavior.

Background: Encouraging results have been recently reported in selected patients affected by pseudomyxoma peritonei (PMP) treated with cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC). The selection factors predicting clinical outcome are still a matter of clinical investigation. We assessed the prognostic reliability of serum tumor markers in a large series of patients with PMP undergoing CRS and HIPEC., Methods: Sixty-two patients with PMP were operated on at a single institution with the intent of performing adequate CRS (residual tumor nodules

Objective: To evaluate the survival of patients with pseudomyxoma peritonei (PMP) treated by cytoreductive surgery and intraoperative hyperthermic intraperitoneal chemotherapy (HIPEC), and to identify factors with prognostic value., Summary Background Data: PMP is a clinical syndrome characterized by progressive intraperitoneal accumulation of mucous and mucinous implants, usually derived from a ruptured mucinous neoplasm of the appendix. Survival is dominated by pathology., Methods: A total of 103 patients (34 men and 69 women) treated at The Netherlands Cancer Institute between 1996 and 2004 were identified. Survival was calculated from date of initial treatment and corrected for a second procedure. PMP was pathologically categorized into disseminated peritoneal adenomucinosis (DPAM), peritoneal mucinous carcinomatosis (PMCA), and an intermediate subtype (PMCA-I). Clinical and pathologic factors were analyzed to identify their prognostic value for survival., Results: Median follow-up was 51.5 months (range, 0.1-99.5 months). Recurrence developed in 44%. A second procedure for recurrence was performed in 11 patients. The median disease-free interval was 25.6 months (95% confidence interval [CI], 14.8-43.6 months). The 3-year and 5-year disease-free survival probability was 43.6% (95% CI, 34.4%-55.2%) and 37.4% (95% CI, 28.2%-49.5%), respectively. The disease-specific 3-year and 5-year survival probability was 70.9% (95% CI, 62.0%-81.2%) and 59.5% (95% CI 48.7%-72.5%), respectively. Factors associated with survival were pathological subtype, completeness of cytoreduction, and degree and location of tumor load (P < 0.05). The main prognostic factor, independently associated with survival, was the pathologic subtype (P < 0.01)., Conclusion: Cytoreductive surgery in combination with intraoperative HIPEC is a feasible treatment strategy for PMP in terms of survival. The pathologic subtype remains the dominant factor in survival. Patients should be centralized to improve survival by a combination of surgical experience and adequate patient selection.

Background: Pseudomyxoma peritonei (PMP) is a rare and fatal disease for which no standard treatment has been established. Encouraging results have been recently reported with the combination of cytoreductive surgery (CRS) and hyperthermic intra-peritoneal chemotherapy (HIPEC)., Patients and Methods: Seventy-five patients with PMP underwent CRS and closed abdomen HIPEC with mytomicin-C and cis-platinum over 10 years at a single institution. Potential clinicopathological prognostic variables were tested using multivariate analysis., Results: Optimal cytoreduction (residual tumor nodules < or = 2.5 mm) was performed in 72 patients (96%). Operative mortality was 1%. Five-year overall (OS) and progression-free (PFS) survival were 78.3% and 31.1% in the overall series, respectively. Optimal CRS, no previous systemic chemotherapy and low histological aggressiveness were independent predictors of better OS and PFS using multivariate analysis., Conclusion: Favourable outcome after CRS and HIPEC can be expected in patients affected by PMP variants with low histological aggressiveness, undergoing optimal surgical cytoreduction and with no pre-operative systemic chemotherapy.

Background: In the literature good results have been reported for the treatment of Pseudomyxoma peritonei (PMP) by cytoreduction, peritonectomy and hyperthermic antiblastic peritoneal perfusion (H.A.P.P.). Forty-eight patients affected by PMP have been treated with this technique over the past ten years., Patients and Methods: Peritoneal perfusion has been performed with the original semiclosed tecnique after complete surgical cytoreduction in 188 patients affected by peritoneal carcinomatosis. In 48 of the cases the patients were affected from PMP. Aggressive surgical cytoreduction was performed with multiple visceral resections and peritonectomies., Results: Seventeen patients (38%) presented major perioperative complications, and in five cases the reoperation of the patient was required. In spite of this high complication rate, there was no perioperative mortality. The results of the Kaplan-Meier 5- and 10-year survival analysis, were 94% and 82%, respectively, with a disease-free survival of 80% at 5 years and 70% at 10 years. Thirty-nine patients (81.2%) had no evidence of disease at follow-up (range 1-120 months)., Discussion: Up to date, the most effective treatment for PMP has been aggressive cytoreduction plus H.A.P.P.

Background: Cytoreductive surgery (CRS) combined with perioperative intraperitoneal chemotherapy (PIC) has been used to treat pseudomyxoma peritonei. The aim of this prospective study was to evaluate survival outcome and treatment-related prognostic markers in patients who underwent CRS and PIC for pseudomyxoma peritonei from appendiceal mucinous neoplasms., Methods: Survival data and 12 clinicopathological and treatment-related prognostic variables for survival were obtained prospectively in 50 consecutive patients (23 men). Univariate analysis was used to determine their prognostic significance for overall survival, determined from the time of CRS., Results: The mean(s.d.) age was 52(12) years. Eighteen patients had moderate complications, and six patients had severe complications that required operation or intensive care support. Two patients died after surgery. The actuarial 5-year survival rate was 69 per cent. Univariate analysis demonstrated that the extent of previous surgery (P = 0.045) and Ronnett's histopathological classification (P < 0.001) were significantly related to overall survival., Conclusion: CRS combined with PIC was associated with improved survival in patients with less extensive previous surgery and diffuse peritoneal adenomucinosis histopathological type.

Pseudomyxoma peritonei is a clinical term for gelatinous ascites, usually secondary to an appendiceal tumor. The pathologic classification of pseudomyxoma peritonei and its associated appendiceal tumors has been plagued with controversy and confusing terminology. In an effort to clarify this, we reviewed the pathology of 101 patients, all treated at our institution from 1993 to 2005, with pseudomyxoma peritonei of appendiceal origin. All patients were uniformly treated with our standardized protocol. This is the largest pathologic series solely devoted to appendiceal neoplasia with gelatinous ascites. The cases were assigned, according to previously published criteria, to the categories of disseminated peritoneal adenomucinosis (DPAM), peritoneal mucinous carcinomatosis (PMCA), or PMCA with intermediate (well differentiated) features (PMCA-I), with the exception that any case with a signet-ring cell component was considered as PMCA and not PMCA-I. By histologic category, 58 patients had DPAM, 23 were PMCA, and 20 were PMCA-I.One-year, 3-year, and 5-year survival outcomes were not significantly different between DPAM and PMCA-I. DPAM and PMCA-I also exhibited a roughly equal incidence of parenchymal (beyond the serosa) organ invasion. Survival outcomes were significantly worse for PMCA, compared with PMCA-I and DPAM. After reviewing our data and the literature, mucinous carcinoma peritonei-low grade was applied to the low-grade histology of pseudomyxoma peritonei, including those cases referred to by some as DPAM in the same category as PMCA-I. Cases that are moderately differentiated to poorly differentiated are classified as mucinous carcinoma peritonei-high grade.

Aims: To report on treatment related toxicity and mortality in patients with pseudomyxoma peritonei (PMP) treated by cytoreduction in combination with intraoperative hyperthermic intraperitoneal chemotherapy (HIPEC) and to identify prognostic factors., Methods: A review was performed of 103 procedures of cytoreduction and intraoperative HIPEC for PMP between 1996 and 2004. Toxicity was graded according to the National Cancer Institute Common Toxicity Criteria (NCI CTC) classification. A surgical complication was defined as any post-operative event that needed re-intervention. Pre and peroperative factors were studied on their relationship to toxicity and mortality., Results: The median hospital stay was 21 days (4-149) with a treatment related toxicity of 54% and a 30 days mortality of 3%. In univariate analysis, toxicity was associated with abdominal tumour load (p<0.01), completeness of cytoreduction (p<0.01), and age (p=0.05). Surgical complications, mainly small bowel perforations/suture leaks, were the main cause of toxicity. A favourable pathology decreased mortality., Conclusions: Cytoreduction in combination with intraoperative HIPEC in PMP patients is a treatment with a relatively high toxicity, but a considerable long-term survival in selected patients. Toxicity is mainly surgery related. Concentration of cases to acquire sufficient experience and better selection on age, pathology, and extent of disease is essential to reduce treatment related toxicity and mortality.

Pseudomyxoma peritonei is an uncommon condition characterized by abundant extracellular mucinous material and peritoneal tumoural implants. A ruptured appendiceal mucinous adenoma seems to be the most common origin, but low grade ovarian malignancies are often associated. Peritoneal gelatinous diseases must be subdivided into disseminated peritoneal adenomucinosis, peritoneal mucinous carcinomatosis and intermediate cases. These entities have significantly different prognosis. Debulking surgery is the most common treatment but some are in favour of an ultra radical surgery with hyperthermic intra-peritoneal chemotherapy, with some encouraging results.

Background and Aims: Surgical improvement can be achieved in selected patients with pseudomyxoma peritonei (PMP) by major cytoreductive surgery and intraperitoneal chemotherapy (IPEC). The purpose of this retrospective study was to analyze morbidity, mortality, and survival following therapy., Patients and Methods: Between July 1995 and September 2003, 28 patients (mean age 56 years, range 28-79) with PMP were operated on with the aim of complete macroscopical cytoreduction. Surgery was followed by IPEC., Results: A macroscopically complete cytoreduction was achieved in 11 patients (40%). The mean operating time was 6 h with a mean of three peritonectomy procedures per patient. Cisplatin (15 out of 28), mitomycin C (6 out of 28) and 5-FU (7 out of 28) were used for the intraoperative chemotherapy. Overall morbidity was 36%. Most frequent surgical complications were digestive fistulae (3 out of 28), abscesses (5 out of 28) and bleeding (2 out of 28). Two patients died postoperatively. Patients with low tumor volume (mean survival time 78+/-11 vs. 37+/-9 months, p=0.05) and complete cytoreduction (73+/-10 vs. 24+/-8 months, p<0.05) had an improved prognosis., Conclusions: Cytoreductive surgery combined with IPEC is associated with acceptable morbidity and mortality. Complete cytoreduction may improve survival, particularly in selected patients with PMP who have a low tumor volume, complete cytoreduction, and no organ metastases.

Summary Background Data: Pseudomyxoma peritonei (PMP) is a clinical syndrome with a poorly defined natural history. Relative contributions of tumor biology, patient selection, and the extent of treatment on ultimate outcome are not well characterized., Methods: Patients treated at the Memorial Sloan-Kettering Cancer Center between 1980 and 2002 with a diagnosis of PMP were identified. Patient characteristics, pathologic features, and details of treatment were analyzed retrospectively., Results: The 97 patients included in this study underwent a mean 2.2 +/- 0.1 operations (range, 1-6). Although complete cytoreduction was achieved in 55% (53/97), disease recurred in 91% (48/53) of patients. The median disease-free interval after complete cytoreduction was 24 months. The median overall survival was 9.8 years and was independently associated with low-grade pathologic subtype (P < 0.001) and the ability to achieve complete cytoreduction (P < 0.001). Ten-year survival was attained in 21% (20/97) of the patients, of which 90% (18/20) had low-grade pathologic features. At the time of death or completion of follow-up, only 12% (12/97) of the patients were disease free., Conclusions: Outcome in patients with PMP is strongly associated with tumor biology. Although improved survival is associated with low-grade pathology and tumors amenable to complete cytoreduction, recurrence of PMP is common. Treatment may be beneficial, particularly in controlling symptoms, but absolute cure, defined as a prolonged disease-free state, is uncommon.

Pseudomyxoma peritonei (PMP) is a rare disease with a poor prognosis when not adequately treated. It is characterized by a complete redistribution of mucin within the peritoneal cavity. The aim of this multicentric study was to evaluate the survival, morbidity, toxicity and mortality of patients with PMP treated by cytoreductive surgery (CRS) with intraperitoneal hyperthermic perfusion (IPHP). Seventy patients with PMP (31 males and 39 females) were enrolled onto a Phase II clinical trial. One patient was operated on twice because of disease recurrence. CRS was performed with peritonectomy procedures. The closed, opened and semi-closed abdomen techniques were employed for IPHP using cisplatin plus mitomycin-C for 60 minutes under hyperthermic conditions (42.5 degrees C). Sixty two (87%) patients were optimally cytoreduced. Five-year overall survival, progression-free survival and locoregional progression-free survival were 91%, 54% and 69%, respectively. Thirteen Grade III complications occurred in 10 (14%) patients and the most frequent one was gastrointestinal fistula/perforation (11%). There was one case (1.4%) of treatment-related mortality 21 days after treatment. CRS associated with IPHP permitted complete tumour removal with an acceptable morbidity and mortality in patients with PMP. This study confirms the efficacy of the combined treatment on long-term survival and local disease control.

PMP is a rare condition, which, although of "borderline malignancy," is invariably fatal. Difficulties exist with the definition of PMP. It has been broadly applied to include a heterogenous group of pathologic lesions that present clinically with "jelly belly" due to mucinous ascites. The relatively few reports in the literature commonly use different pathologic definitions, and there is no consensus on the point of separation between PMP and carcinomatosis secondary to a mucinous adenocarcinoma. Sugarbaker has suggested "the term pseudomyxoma peritonei syndrome be strictly applied to a pathologically and prognostically homogenous group of cases characterized by histologically benign peritoneal tumors that are frequently associated with an appendiceal mucinous adenoma." This definition excludes all cases with mucinous adenocarcinoma. The optimal treatment is undoubtedly complete tumor excision, by complex surgical peritonectomy procedures, taking on average 10 hours. Surgery is usually combined with intraperitoneal, and now intraoperative heated chemotherapy. These techniques have a high morbidity and mortality. The rarity of the condition, together with the risks associated with definitive treatment, suggests that such treatment ought to be centralized in a few centers, covering a large population. The search continues for safer, less aggressive treatments, but is hampered by a lack of hard evidence and the absence of experimental animal or human models to evaluate emerging strategies.

Pseudomyxoma peritonei is a rare disease characterized by a complete redistribution of mucin into the peritoneal cavity. Thirty three PMP patients referred to National Cancer Institute of Milan were submitted to 34 consecutive procedures of peritonectomy and intra peritoneal hyperthermic perfusion (IPHP). Five yrs overall survival has been 96% while 3 yrs PFS 68%. Morbility, mortality and toxicity were 18, 3 and 9% respectively. Peritonectomy associated with IPHP is the most indicated approach to cure patients with DPAM and IFG, subgroup of pseudomyxoma peritonei.

Background: Tumor markers are useful for diagnosis and follow-up. We studied the prognostic value of baseline and serial carcinoembryonic antigen (CEA) and carbohydrate antigen 19.9 (CA19.9) measurements in patients with pseudomyxoma peritonei treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC)., Methods: Sixty-three patients with pseudomyxoma peritonei were treated with cytoreductive surgery and HIPEC. The tumor markers CEA and CA19.9 were collected before therapy and at 3-month intervals during follow-up., Results: Preoperative CEA and CA19.9 levels were increased in, respectively, 75% and 58% of the patients. Baseline tumor marker values were related to the extent of tumor. Immediately after HIPEC, both tumor markers decreased markedly (P <.0001). CA19.9 was shown to be a more useful tumor marker than CEA for follow-up. During follow-up, a high absolute CA19.9 level (P =.0005) was predictive for imminent recurrence. Patients who never attained a normal CA19.9 level showed a higher recurrence rate at 1 year (53%; SE, 15%), in comparison to patients who did so (6%; SE 4%). The median lead time of increased CA19.9 to recurrence was 9 months., Conclusions: The measurement of the tumor marker CA19.9 is useful in evaluating therapy in patients with pseudomyxoma peritonei treated with cytoreductive surgery and HIPEC. CA19.9 is a prognostic factor for predicting recurrent disease.

Background/aims: Pseudomyxoma peritonei is a rare neoplasm with intra-abdominal gelatinous, jelly-like fluid accumulation. To assess the clinical characteristics, treatment modalities, and predictors of survival of pseudomyxoma peritonei in Chinese patients, we conducted this study., Methodology: Over a 27-year period, 15 patients with the diagnosis of pseudomyxoma peritonei were enrolled. Their demographic, clinical, treatment and survival data were collected., Results: Seven patients were female and eight were male. The median age at diagnosis was 48 years old for 7 females and 66 years old for 8 males. Exploratory laparotomy and debulking surgery were performed for 11 patients (73%); 6 of these 11 patients received adjuvant chemotherapy. Among 4 patients without surgical treatment, 1 received chemotherapy and 3 had supportive treatment only. The 5-year survival for all patients and patients with surgical treatment were 39% and 62%, respectively. Patients with surgical intervention had longer survival (median, 125 months) than those without surgical treatment (median, 8 months) (P = 0.0106). However, adjuvant chemotherapy did not further prolong the survival time (P = 0.744)., Conclusions: Surgical intervention rather than adjuvant chemotherapy appears to provide a longer survival for patients with pseudomyxoma peritonei.

Aims: If mucinous appendiceal tumours widely distributed throughout the peritoneal space are completely removed by peritonectomy procedures, quality of life and survival benefits result with an acceptable morbidity and mortality. In some patients mucinous tumour that surrounds the stomach is an important consideration in accomplishing a complete cytoreduction., Methods: Mucinous tumour that enters the lesser sac through the foramen of Winslow will accumulate by gravity in the subpyloric space. This is a cul-de-sac beneath the pylorus. For complete cytoreduction mucinous tumour accumulation in the subpyloric space must be cleared., Results: If there is tumour accumulation in the subpyloric space and the left gastric artery can be preserved by peritonectomy, one can achieve a complete cytoreduction without gastrectomy. In a majority of patients, resection of mucinous tumour from the subpyloric space requires total gastrectomy for complete cytoreduction., Conclusion: Knowledge of mucinous tumour distribution and anatomy of the subpyloric space will facilitate complete cytoreduction in selected patients with pseudomyxoma peritonei syndrome.