UpToDate
Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Medline ® Abstracts for References 30,41,45-51

of 'Cancer of the appendix and pseudomyxoma peritonei'

30
TI
Pseudomyxoma peritonei: clinical pathological and biological prognostic factors in patients treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC).
AU
Baratti D, Kusamura S, Nonaka D, Langer M, Andreola S, Favaro M, Gavazzi C, Laterza B, Deraco M
SO
Ann Surg Oncol. 2008;15(2):526. Epub 2007 Nov 28.
 
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.
AD
Department of Surgery, National Cancer Institute, Milan, Italy.
PMID
41
TI
Results of treatment of 385 patients with peritoneal surface spread of appendiceal malignancy.
AU
Sugarbaker PH, Chang D
SO
Ann Surg Oncol. 1999;6(8):727.
 
INTRODUCTION: In the past, peritoneal carcinomatosis, regardless of primary tumor type, has always been a lethal condition. Recently, special treatments using cytoreductive surgery with peritonectomy procedures combined with perioperative intraperitoneal chemotherapy have resulted in long-term survival. Appendiceal malignancy with a low incidence of liver and lymph node metastases may be especially appropriate for these aggressive local regional treatments.
METHODS: All patients treated with surgery before January 1999 are included. Patients left with gross residual disease after surgery were not given intraperitoneal chemotherapy, but were later treated with intravenous chemotherapy. The intraperitoneal chemotherapy was given in the perioperative period, starting with mitomycin C at 12.5 mg/m2 for males and 10 mg/m2 for females. For patients whose pathology showed adenomucinosis, intraperitoneal chemotherapy was limited to treatment in the operating theater with heated mitomycin C. Patients with mucinous adenocarcinoma or pseudomyxoma/adenocarcinoma hybrid had, in addition to mitomycin C, five consecutive days of intraperitoneal 5-fluorouracil at 650 mg/m2 instilled in 1-1.5 liters of 1.5% dextrose peritoneal dialysis solution. A complete cytoreduction was defined as tumor nodules<2.5 mm in diameter remaining after surgery. Thehistopathology categorized the patients as having adenomucinosis, adenomucinosis/carcinomatosis hybrid, or mucinous carcinomatosis. A previous surgical score was used to estimate the extent of previous surgical procedures.
RESULTS: The morbidity of treated patients was 27% and the mortality was 2.7%. In a multivariate analysis, prognostic factors for survival included the completeness of cytoreduction (P<.0001), the histopathological character of the appendix malignancy (P<.0001), and the extent of previous surgical interventions (P = .001). Patients with a complete cytoreduction and adenomucinosis by pathology had a 5-year survival of 86%; with hybrid pathology, survival at 5 years was 50%. Incomplete cytoreduction had a 5-year survival of 20% and 0% at 10 years.
CONCLUSIONS: Cytoreductive surgery and perioperative intraperitoneal chemotherapy can be used to salvage selected patients with peritoneal surface spread of appendiceal primary tumors. Similar strategies for other patients with peritoneal surface malignancy such as peritoneal carcinomatosis from colon or gastric cancer, peritoneal sarcomatosis, or peritoneal mesothelioma should be pursued.
AD
Washington Cancer Institute, Washington, DC 20010, USA.
PMID
45
TI
Appendiceal neoplasms with peritoneal dissemination: outcomes after cytoreductive surgery and intraperitoneal hyperthermic chemotherapy.
AU
Stewart JH 4th, Shen P, Russell GB, Bradley RF, Hundley JC, Loggie BL, Geisinger KR, Levine EA
SO
Ann Surg Oncol. 2006;13(5):624. Epub 2006 Mar 14.
 
BACKGROUND: Appendiceal neoplasms frequently present with peritoneal dissemination (PD) and have a clinical course marked by bowel obstruction and subsequent death. Few data have correlated outcome with appendiceal histology after cytoreductive surgery and intraperitoneal hyperthermic chemotherapy (IPHC). We have reviewed our experience with cytoreductive surgery and IPHC for PD from the appendix.
METHODS: A total of 110 cases of PD from proven appendiceal neoplasms treated with IPHC were identified from a prospectively managed database. Tumor samples were classified on pathologic review as disseminated peritoneal adenomucinosis (n = 55), peritoneal mucinous carcinomatosis (PMCA) with intermediate features (n = 18), PMCA (n = 29), or high-grade nonmucinous lesions (n = 8). A retrospective review was performed with long-term survival as the primary outcome measure.
RESULTS: A total of 116 IPHCs were performed on 110 patients for appendiceal PD between 1993 and 2004. The 1-, 3-, and 5-year survival rates for all cases were 79.9% +/- 4.1%, 59.0% +/- 5.7%, and 53.4% +/- 6.5%, respectively. When stratified by histology, disseminated peritoneal adenomucinosis and intermediate tumors had better 3-year survival rates (77% +/- 7% and 81% +/- 10%) than PMCA and high-grade nonmucinous lesions (35% +/- 10% and 15% +/- 14%; P = .0032 for test of differences between groups). Age at presentation (P = .0134), performance status (P<.0001), time between diagnosis and IPHC (P = .0011), resection status (P = .0044), and length of hyperthermic chemoperfusion (P = .0193) were independently associated with survival.
CONCLUSIONS: The data show that long-term survival is anticipated in most patients who are treated with cytoreduction and IPHC for appendiceal PD. The findings presented herein underscore the important prognostic characteristics that predict outcome after IPHC in patients with PD. In all, this work establishes a framework for the consideration of IPHC in future trials for appendiceal PD.
AD
Department of General Surgery, Surgical Oncology Service, Wake Forest University School of Medicine, Medical Center Boulevard, Winston-Salem, North Carolina 27157, USA.
PMID
46
TI
[Pseudomyxoma peritonei: new concept and new therapeutic approach].
AU
Loungnarath R, Causeret S, Brigand C, Gilly FN, Glehen O
SO
Ann Chir. 2005;130(2):63.
 
Pseudomyxoma peritonei is a rare disease, usually diagnosed after the discover of "jelly belly" by laparotomy. With the progress of immunohistochemistry, most authors now acknowledge the appendix to be the principal origin of this disease. Pseudomyxoma peritonei need to considered as border line malignant disease because of its inevitable persistence and progression without adapted therapeutic approach: cytoreductive surgery combined with perioperative intraperitoneal chemotherapy (intraperitoneal chemohyperthermia and/or immediate postoperative intraperitoneal chemotherapy) into specialized centres. The principal prognostic factors are the prior surgical history, the completeness of cytoreduction and especially the histopathologic grade.
AD
Département de chirurgie colorectale, centre hospitalier de l'universitéde montréal, hôpital Saint-Luc, Montréal, Québec, Canada.
PMID
47
TI
Operability and early outcome in 100 consecutive laparotomies for peritoneal malignancy.
AU
Moran BJ, Mukherjee A, Sexton R
SO
Br J Surg. 2006;93(1):100.
 
BACKGROUND: Peritoneal malignancy is common at the terminal stages of many intra-abdominal neoplasms. In selected patients a novel approach of complete macroscopic tumour removal by extensive surgical cytoreduction, combined with intraperitoneal chemotherapy, provides a possibility of cure. Initial experience with 100 consecutive laparotomies is reported.
METHODS: Between 1994 and 2002, 218 patients with peritoneal malignancy were referred to a specialized unit. One hundred underwent laparotomy, of whom 85 had a primary appendiceal tumour. Treatment aims were complete macroscopic tumour excision combined with intraoperative intraperitoneal chemotherapy.
RESULTS: Sixty-five patients had complete macroscopic tumour excision, 28 palliative major cytoreduction and seven were inoperable. The overall mortality rate was 8 per cent. The most common complications were pulmonary (25 per cent) and wound (14 per cent) infection. At a median follow-up of 30 (range 3-113) months, 44 of 51 patients with primary appendiceal tumours who had complete macroscopic cytoreduction were alive and 36 were disease free.
CONCLUSION: Cytoreduction combined with intraperitoneal chemotherapy is an emerging technique with promising results in selected patients with peritoneal malignancy.
AD
Pseudomyxoma Peritonei Centre, North Hampshire Hospital, Aldermaston Road, Basingstoke RG24 9NA, UK. Brendan.Moran@nhht.nhs.uk
PMID
48
TI
Cytoreductive surgery and intraperitoneal chemotherapy for pseudomyxoma peritonei.
AU
Güner Z, Schmidt U, Dahlke MH, Schlitt HJ, Klempnauer J, Piso P
SO
Int J Colorectal Dis. 2005;20(2):155. Epub 2004 Oct 16.
 
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.
AD
Department for Visceral and Transplant Surgery, Medical School of Hannover, 30623 Hannover, Germany.
PMID
49
TI
Long-term survival in patients with pseudomyxoma peritonei treated with cytoreductive surgery and perioperative intraperitoneal chemotherapy: 10 years of experience from a single institution.
AU
Chua TC, Yan TD, Smigielski ME, Zhu KJ, Ng KM, Zhao J, Morris DL
SO
Ann Surg Oncol. 2009;16(7):1903.
 
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 was104 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.
AD
Department of Surgery, St George Hospital, University of New South Wales, Sydney, NSW, Australia. chua.terence@yahoo.com.sg
PMID
50
TI
Operative findings, early complications, and long-term survival in 456 patients with pseudomyxoma peritonei syndrome of appendiceal origin.
AU
Youssef H, Newman C, Chandrakumaran K, Mohamed F, Cecil TD, Moran BJ
SO
Dis Colon Rectum. 2011;54(3):293.
 
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.
AD
Basingstoke and North Hampshire NHS Foundation Trust, Basingstoke, UK.
PMID
51
TI
Pseudomyxoma peritonei: a French multicentric study of 301 patients treated with cytoreductive surgery and intraperitoneal chemotherapy.
AU
Elias D, Gilly F, Quenet F, Bereder JM, Sidéris L, Mansvelt B, Lorimier G, Glehen O, Association Française de Chirurgie
SO
Eur J Surg Oncol. 2010;36(5):456. Epub 2010 Mar 12.
 
OBJECTIVE: To analyze a large series of patients with pseudomyxoma peritonei (PMP) treated with cytoreductive surgery associated with perioperative intraperitoneal chemotherapy (PIC) in 18 French-speaking centers.
PATIENTS AND METHODS: From March 1993 to December 2007, 301 patients with diffuse PMP were treated by cytoreductive surgery with PIC. Complete cytoreductive surgery was achieved in 219 patients (73%), and hyperthermic intraperitoneal chemotherapy (HIPEC) was performed in 255 (85%), mainly during the latter period of the study.
RESULTS: Postoperative mortality and morbidity were 4.4% and 40%, respectively. The mean follow-up was 88 months. The 5-year overall and disease-free survival rates were 73% and 56%, respectively. The multivariate analysis identified 5 prognostic factors: the extent of peritoneal seeding (p=0.004), the center (p=0.0004), the pathologic grade (p=0.03), gender (p=0.02), and the use of HIPEC (p=0.04). When only the 206 patients with complete cytoreductive surgery were considered, the extent of peritoneal seeding was the only significant prognostic factor (p=0.004).
CONCLUSION: This large multicentric retrospective study confirms that cytoreductive surgery combined with PIC (with the use of hyperthermia) should be considered as the gold standard treatment of PMP and should be performed in specialized centers. It underlines the prognostic impact of the extent of peritoneal seeding, especially in patients treated by complete cytoreductive surgery. This prognostic impact appears to be greater than that of the pathologic grade.
AD
Department of Oncologic Surgery, Institut Gustave Roussy, 39 Rue Camille Desmoulins, 94805 Villejuif Cédex, France. elias@igr.fr
PMID