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Published in: Annals of Surgical Oncology 7/2009

01-07-2009 | Gastrointestinal Oncology

Long-Term Survival in Patients with Pseudomyxoma Peritonei Treated with Cytoreductive Surgery and Perioperative Intraperitoneal Chemotherapy: 10 Years of Experience from a Single Institution

Authors: Terence C. Chua, BScMed (Hons), Tristan D. Yan, PhD, Michelle E. Smigielski, Katherine J. Zhu, MBBS, Keh M. Ng, MBBS, Jing Zhao, MD, David L. Morris, MD

Published in: Annals of Surgical Oncology | Issue 7/2009

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Abstract

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.
Literature
1.
go back to reference Moran BJ, Cecil TD. The etiology, clinical presentation, and management of pseudomyxoma peritonei. Surg Oncol Clin N Am. 2003;12:585-603.PubMedCrossRef Moran BJ, Cecil TD. The etiology, clinical presentation, and management of pseudomyxoma peritonei. Surg Oncol Clin N Am. 2003;12:585-603.PubMedCrossRef
2.
go back to reference Ronnett BM, Zahn CM, Kurman RJ, Kass ME, Sugarbaker PH, Shmookler BM. Disseminated peritoneal adenomucinosis and peritoneal mucinous carcinomatosis A clinicopathologic analysis of 109 cases with emphasis on distinguishing pathologic features, site of origin, prognosis, and relationship to “pseudomyxoma peritonei.” Am J Surg Pathol. 1995;19:1390–408.PubMed Ronnett BM, Zahn CM, Kurman RJ, Kass ME, Sugarbaker PH, Shmookler BM. Disseminated peritoneal adenomucinosis and peritoneal mucinous carcinomatosis A clinicopathologic analysis of 109 cases with emphasis on distinguishing pathologic features, site of origin, prognosis, and relationship to “pseudomyxoma peritonei.” Am J Surg Pathol. 1995;19:1390–408.PubMed
3.
go back to reference Yan TD, Links M, Xu ZY, Kam PC, Glenn D, Morris DL. Cytoreductive surgery and perioperative intraperitoneal chemotherapy for pseudomyxoma peritonei from appendiceal mucinous neoplasms. Br J Surg. 2006;93:1270–6.PubMedCrossRef Yan TD, Links M, Xu ZY, Kam PC, Glenn D, Morris DL. Cytoreductive surgery and perioperative intraperitoneal chemotherapy for pseudomyxoma peritonei from appendiceal mucinous neoplasms. Br J Surg. 2006;93:1270–6.PubMedCrossRef
4.
go back to reference Baratti D, Kusamura S, Nonaka D, et al. Pseudomyxoma peritonei: clinical pathological and biological prognostic factors in patients treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC). Ann Surg Oncol. 2008;15:526–34.PubMedCrossRef Baratti D, Kusamura S, Nonaka D, et al. Pseudomyxoma peritonei: clinical pathological and biological prognostic factors in patients treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC). Ann Surg Oncol. 2008;15:526–34.PubMedCrossRef
5.
go back to reference Ronnett BM, Yan H, Kurman RJ, Shmookler BM, Wu L, Sugarbaker PH. Patients with pseudomyxoma peritonei associated with disseminated peritoneal adenomucinosis have a significantly more favorable prognosis than patients with peritoneal mucinous carcinomatosis. Cancer. 2001;92:85–91.PubMedCrossRef Ronnett BM, Yan H, Kurman RJ, Shmookler BM, Wu L, Sugarbaker PH. Patients with pseudomyxoma peritonei associated with disseminated peritoneal adenomucinosis have a significantly more favorable prognosis than patients with peritoneal mucinous carcinomatosis. Cancer. 2001;92:85–91.PubMedCrossRef
6.
go back to reference Smith JW, Kemeny N, Caldwell C, Banner P, Sigurdson E, Huvos A. Pseudomyxoma peritonei of appendiceal origin. The Memorial Sloan-Kettering Cancer Center experience. Cancer. 1992;70:396–401.PubMedCrossRef Smith JW, Kemeny N, Caldwell C, Banner P, Sigurdson E, Huvos A. Pseudomyxoma peritonei of appendiceal origin. The Memorial Sloan-Kettering Cancer Center experience. Cancer. 1992;70:396–401.PubMedCrossRef
7.
go back to reference Nitecki SS, Wolff BG, Schlinkert R, Sarr MG. The natural history of surgically treated primary adenocarcinoma of the appendix. Ann Surg. 1994;219:51–7.PubMedCrossRef Nitecki SS, Wolff BG, Schlinkert R, Sarr MG. The natural history of surgically treated primary adenocarcinoma of the appendix. Ann Surg. 1994;219:51–7.PubMedCrossRef
8.
go back to reference Gough DB, Donohue JH, Schutt AJ, et al. Pseudomyxoma peritonei. Long-term patient survival with an aggressive regional approach. Ann Surg. 1994;219:112–9.PubMedCrossRef Gough DB, Donohue JH, Schutt AJ, et al. Pseudomyxoma peritonei. Long-term patient survival with an aggressive regional approach. Ann Surg. 1994;219:112–9.PubMedCrossRef
9.
go back to reference Miner TJ, Shia J, Jaques DP, Klimstra DS, Brennan MF, Coit DG. Long-term survival following treatment of pseudomyxoma peritonei: an analysis of surgical therapy. Ann Surg. 2005;241:300–8.PubMedCrossRef Miner TJ, Shia J, Jaques DP, Klimstra DS, Brennan MF, Coit DG. Long-term survival following treatment of pseudomyxoma peritonei: an analysis of surgical therapy. Ann Surg. 2005;241:300–8.PubMedCrossRef
10.
go back to reference Sugarbaker PH. New standard of care for appendiceal epithelial neoplasms and pseudomyxoma peritonei syndrome? Lancet Oncol. 2006;7:69–76.PubMedCrossRef Sugarbaker PH. New standard of care for appendiceal epithelial neoplasms and pseudomyxoma peritonei syndrome? Lancet Oncol. 2006;7:69–76.PubMedCrossRef
11.
go back to reference Yan TD, Black D, Savady R, Sugarbaker PH. A systematic review on the efficacy of cytoreductive surgery and perioperative intraperitoneal chemotherapy for pseudomyxoma peritonei. Ann Surg Oncol. 2007;14:484–92.PubMedCrossRef Yan TD, Black D, Savady R, Sugarbaker PH. A systematic review on the efficacy of cytoreductive surgery and perioperative intraperitoneal chemotherapy for pseudomyxoma peritonei. Ann Surg Oncol. 2007;14:484–92.PubMedCrossRef
12.
go back to reference Jacquet P, Sugarbaker PH. Current methodologies for clinical assessment of patients with peritoneal carcinomatosis. J Exp Clin Cancer Res. 1996;15:49–58. Jacquet P, Sugarbaker PH. Current methodologies for clinical assessment of patients with peritoneal carcinomatosis. J Exp Clin Cancer Res. 1996;15:49–58.
14.
go back to reference Yan TD, Bijelic L, Sugarbaker PH. Critical analysis of treatment failure after complete cytoreductive surgery and perioperative intraperitoneal chemotherapy for peritoneal dissemination from appendiceal mucinous neoplasms. Ann Surg Oncol. 2007;14:2289–99.PubMedCrossRef Yan TD, Bijelic L, Sugarbaker PH. Critical analysis of treatment failure after complete cytoreductive surgery and perioperative intraperitoneal chemotherapy for peritoneal dissemination from appendiceal mucinous neoplasms. Ann Surg Oncol. 2007;14:2289–99.PubMedCrossRef
15.
go back to reference Smeenk RM, Verwaal VJ, Antonini N, Zoetmulder FAN. Survival analysis of pseudomyxoma peritonei patients treated by cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. Ann Surg. 2007;245:104–9.PubMedCrossRef Smeenk RM, Verwaal VJ, Antonini N, Zoetmulder FAN. Survival analysis of pseudomyxoma peritonei patients treated by cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. Ann Surg. 2007;245:104–9.PubMedCrossRef
16.
go back to reference Elias D, Honore C, Ciuchendea R, et al. Peritoneal pseudomyxoma: results of a systematic policy of complete cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. Br J Surg. 2008;95:1164–71.PubMedCrossRef Elias D, Honore C, Ciuchendea R, et al. Peritoneal pseudomyxoma: results of a systematic policy of complete cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. Br J Surg. 2008;95:1164–71.PubMedCrossRef
17.
go back to reference Sugarbaker PH, Chang D. Results of treatment of 385 patients with peritoneal surface spread of appendiceal malignancy. Ann Surg Oncol. 1999;6:727–31.PubMedCrossRef Sugarbaker PH, Chang D. Results of treatment of 385 patients with peritoneal surface spread of appendiceal malignancy. Ann Surg Oncol. 1999;6:727–31.PubMedCrossRef
18.
go back to reference de Melo GM, Ribeiros KC, Kowalski LP, Deheinzelin D. Risk factors for postoperative complications in oral cancer and their prognostic implications. Arch Otolaryngol Head Neck Surg. 2001;127:823–33. de Melo GM, Ribeiros KC, Kowalski LP, Deheinzelin D. Risk factors for postoperative complications in oral cancer and their prognostic implications. Arch Otolaryngol Head Neck Surg. 2001;127:823–33.
19.
go back to reference Fujita S, Teramoto T, Watanabe M, Kodaira S, Kitajima M. Anastomotic leakage after colorectal surgery: a risk factor for recurrence and poor prognosis. Jpn J Clin Oncol. 1993;23:299–302.PubMed Fujita S, Teramoto T, Watanabe M, Kodaira S, Kitajima M. Anastomotic leakage after colorectal surgery: a risk factor for recurrence and poor prognosis. Jpn J Clin Oncol. 1993;23:299–302.PubMed
20.
go back to reference Hirai T, Yamashita Y, Mukaida H, Kuwahara M, Inoue H, Toge T. Poor prognosis in esophageal cancer patients with postoperative complications. Surg Today. 1998;28:576–9.PubMedCrossRef Hirai T, Yamashita Y, Mukaida H, Kuwahara M, Inoue H, Toge T. Poor prognosis in esophageal cancer patients with postoperative complications. Surg Today. 1998;28:576–9.PubMedCrossRef
21.
go back to reference Kressner U, Graf W, Mahteme H, Pahlman L, Glimelius B. Septic complications and prognosis after surgery for rectal cancer. Dis Colon Rectum. 2002;45:316–21.PubMedCrossRef Kressner U, Graf W, Mahteme H, Pahlman L, Glimelius B. Septic complications and prognosis after surgery for rectal cancer. Dis Colon Rectum. 2002;45:316–21.PubMedCrossRef
22.
go back to reference Nespoli A, Gianotti L, Bovo G, Brivio F, Nespoli L, Totis M. Impact of postoperative infections on survival in colon cancer patients. Surg Infect. 2006;7(Suppl 2):S41–3. Nespoli A, Gianotti L, Bovo G, Brivio F, Nespoli L, Totis M. Impact of postoperative infections on survival in colon cancer patients. Surg Infect. 2006;7(Suppl 2):S41–3.
23.
go back to reference Kraft A, Weindel K, Ochs A, et al. Vascular endothelial growth factor in the sera and effusions of patients with malignant and nonmalignant disease. Cancer. 1999;85:178–87.PubMedCrossRef Kraft A, Weindel K, Ochs A, et al. Vascular endothelial growth factor in the sera and effusions of patients with malignant and nonmalignant disease. Cancer. 1999;85:178–87.PubMedCrossRef
24.
go back to reference Ronnett BM, Shmookler BM, Diener-West M, Sugarbaker PH, Kurman RJ. Immunohistochemical evidence supporting the appendiceal origin of pseudomyxoma peritonei in women. Int J Gynecol Pathol. 1997;16:1–9.PubMed Ronnett BM, Shmookler BM, Diener-West M, Sugarbaker PH, Kurman RJ. Immunohistochemical evidence supporting the appendiceal origin of pseudomyxoma peritonei in women. Int J Gynecol Pathol. 1997;16:1–9.PubMed
25.
go back to reference Szych C, Staebler A, Connolly DC, Wu R, Cho KR, Ronnett BM. Molecular genetic evidence supporting the clonality and appendiceal origin of Pseudomyxoma peritonei in women. Am J Pathol. 1999;154:1849–55.PubMed Szych C, Staebler A, Connolly DC, Wu R, Cho KR, Ronnett BM. Molecular genetic evidence supporting the clonality and appendiceal origin of Pseudomyxoma peritonei in women. Am J Pathol. 1999;154:1849–55.PubMed
26.
go back to reference Maheshwari V, Tsung A, Lin Y, Zeh HJ 3rd, Finkelstein SD, Bartlett DL. Analysis of loss of heterozygosity for tumor-suppressor genes can accurately classify and predict the clinical behavior of mucinous tumors arising from the appendix. Ann Surg Oncol. 2006;13:1610–6.PubMedCrossRef Maheshwari V, Tsung A, Lin Y, Zeh HJ 3rd, Finkelstein SD, Bartlett DL. Analysis of loss of heterozygosity for tumor-suppressor genes can accurately classify and predict the clinical behavior of mucinous tumors arising from the appendix. Ann Surg Oncol. 2006;13:1610–6.PubMedCrossRef
27.
go back to reference Logan-Collins JM, Lowy AM, Robinson-Smith TM, et al. VEGF expression predicts survival in patients with peritoneal surface metastases from mucinous adenocarcinoma of the appendix and colon. Ann Surg Oncol. 2008;15:738–44.PubMedCrossRef Logan-Collins JM, Lowy AM, Robinson-Smith TM, et al. VEGF expression predicts survival in patients with peritoneal surface metastases from mucinous adenocarcinoma of the appendix and colon. Ann Surg Oncol. 2008;15:738–44.PubMedCrossRef
28.
go back to reference Semino-Mora C, Liu H, McAvoy T, et al. Pseudomyxoma peritonei: is disease progression related to microbial agents? A study of bacteria, MUC2 AND MUC5AC expression in disseminated peritoneal adenomucinosis and peritoneal mucinous carcinomatosis. Ann Surg Oncol. 2008;15:1414–23.PubMedCrossRef Semino-Mora C, Liu H, McAvoy T, et al. Pseudomyxoma peritonei: is disease progression related to microbial agents? A study of bacteria, MUC2 AND MUC5AC expression in disseminated peritoneal adenomucinosis and peritoneal mucinous carcinomatosis. Ann Surg Oncol. 2008;15:1414–23.PubMedCrossRef
29.
go back to reference O’Connell JT, Hacker CM, Barsky SH. MUC2 is a molecular marker for pseudomyxoma peritonei. Mod Pathol. 2002;15:958–72.PubMedCrossRef O’Connell JT, Hacker CM, Barsky SH. MUC2 is a molecular marker for pseudomyxoma peritonei. Mod Pathol. 2002;15:958–72.PubMedCrossRef
30.
go back to reference Nonaka D, Kusamura S, Baratti D, Casali P, Younan R, Deraco M. CDX-2 expression in pseudomyxoma peritonei: a clinicopathological study of 42 cases. Histopathology. 2006;49:381–7.PubMedCrossRef Nonaka D, Kusamura S, Baratti D, Casali P, Younan R, Deraco M. CDX-2 expression in pseudomyxoma peritonei: a clinicopathological study of 42 cases. Histopathology. 2006;49:381–7.PubMedCrossRef
31.
go back to reference Heiskala K, Giles-Komar J, Heiskala M, Andersson LC. High expression of RELP (Reg IV) in neoplastic goblet cells of appendiceal mucinous cystadenoma and pseudomyxoma peritonei. Virch Arch. 2006;448:295–300.CrossRef Heiskala K, Giles-Komar J, Heiskala M, Andersson LC. High expression of RELP (Reg IV) in neoplastic goblet cells of appendiceal mucinous cystadenoma and pseudomyxoma peritonei. Virch Arch. 2006;448:295–300.CrossRef
32.
go back to reference Bibi R, Pranesh N, Saunders MP, et al. A specific cadherin phenotype may characterise the disseminating yet non-metastatic behaviour of pseudomyxoma peritonei. Br J Cancer. 2006;95:1258–64.PubMedCrossRef Bibi R, Pranesh N, Saunders MP, et al. A specific cadherin phenotype may characterise the disseminating yet non-metastatic behaviour of pseudomyxoma peritonei. Br J Cancer. 2006;95:1258–64.PubMedCrossRef
33.
go back to reference Farquharson AL, Pranesh N, Witham G, et al. A phase II study evaluating the use of concurrent mitomycin C and capecitabine in patients with advanced unresectable pseudomyxoma peritonei. Br J Cancer. 2008;99:591–6.PubMedCrossRef Farquharson AL, Pranesh N, Witham G, et al. A phase II study evaluating the use of concurrent mitomycin C and capecitabine in patients with advanced unresectable pseudomyxoma peritonei. Br J Cancer. 2008;99:591–6.PubMedCrossRef
Metadata
Title
Long-Term Survival in Patients with Pseudomyxoma Peritonei Treated with Cytoreductive Surgery and Perioperative Intraperitoneal Chemotherapy: 10 Years of Experience from a Single Institution
Authors
Terence C. Chua, BScMed (Hons)
Tristan D. Yan, PhD
Michelle E. Smigielski
Katherine J. Zhu, MBBS
Keh M. Ng, MBBS
Jing Zhao, MD
David L. Morris, MD
Publication date
01-07-2009
Publisher
Springer-Verlag
Published in
Annals of Surgical Oncology / Issue 7/2009
Print ISSN: 1068-9265
Electronic ISSN: 1534-4681
DOI
https://doi.org/10.1245/s10434-009-0341-8

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