Abstract
Background
Pseudomyxoma peritonei (PMP) is a rare disease with an incidence rate of approximately 1 per million a year. During the past few years, there has been a survival benefit for these patients treated by complete cytoreduction and perioperative chemotherapy. Better survival rates were found in the adenomucinosis group than the carcinomatosis group. The purpose of our study was to analyze the outcome and the prognosis factors of only high-grade PMP.
Methods
We selected 38 patients from a prospective database of 59 with high-grade PMP from appendiceal origin who were treated by cytoreduction surgery and HIPEC at the Hospital University Reina Sofia (Cordoba, Spain) between 1998 and July 2012. Clinical, surgical, analytical, radiological, and histological data were obtained prospectively. Survival curves were calculated using the Kaplan–Meier method, a univariate analysis was performed and the log rank-test was used to analyze the effects of several clinical and pathologic factors on overall survival (OS) and disease-free survival (DFS).
Results
Median follow-up time was 32 months (range, 2–170). Median age at diagnosis was 57 years (range, 32–77). In 89.5 % of patients, optimal cytoreduction CC-0 (57.9 %) and CC-1 (31.6 %) was achieved. In the remaining 10.5 %, cytoreduction was classified as CC-2. The median PCI score was 21 (range, 4–38). Morbidity complications ≥Grade 3 in the CTCAE v 3.0 classification was 18.4 %. One patient died 45 days postsurgery. Median OS at the end of follow-up was 36 months (range, 9–83); overall 5-year survival rate was 58.7 %. In the univariate analysis for OS, significant values were obtained for lymph-node involvement and suboptimal cytoreduction. The 5-year OS was 64.5 % when an optimal cytoreduction was achieved. Median DFS was 36 months (17–54); 3-year DFS rate was 49.1 %. Neoadjuvant therapy did not affect the survival of these patients; there was no difference in the 5-year OS (43 % vs. 75 %, p = 0.068).
Conclusions
In aggressive PMP, cytoreduction with peritonectomy procedure plus HIPEC is a safe procedure that suggests an improvement to the survival rates. Because optimal cytoreduction is a primary prognostic factor for survival rates, this procedure would have to be performed in an experienced center with a low morbidity. Neoadjuvant chemotherapy has not demonstrated benefits in these patients and further research will be required.
Similar content being viewed by others
References
Smeenk RM, Verwaal VJ, Antonini N, Zoetmulder FAN (2007) Survival analysis of Pseudomyxoma peritonei patients treated by cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. Ann Surg 245:104–109
Moran B, Baratti D, Yan TD, Kasamura S, Deraco M (2008) Consensus statement on the loco-regional treatment of appendiceal mucinous neoplasms with peritoneal dissemination (Pseudomyxoma peritonei). J Surg Oncol 98:277–282
Sugarbaker PH (1995) Peritonectomy procedure. Ann Surg 221:29–42
Ronnett BM, Kurman RJ, Zahn CM et al (1995) Pseudomyxoma peritonei in women: a clinicopathologic analysis of 30 cases with emphasis on site of origin, prognosis and relationship to ovarian mucinous tumors of low malignant potential. Hum Pathol 26:509–524
Chua TC, Moran BJ, Sugarbaker PH et al (2012) Early and long-term outcome data of patients with Pseudomyxoma peritonei from appendiceal origin treated by a strategy of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. J Clin Oncol 30(20):2449–2456
Bruin SC, Verwaal VJ, Vincent A, van’t Veer LJ, van Velthuysen ML (2010) A clinicopathologic analysis of peritoneal metastases of colorectal and appendiceal origin. Ann Surg Oncol 17:2330–2340
Sorensen O, Flatmark K, Reed W, Wiig JN, Dueland S, Giercksky K, Larsen SG (2012) Evaluation of complete cytoreductive surgery and two intraperitoneal chemotherapy techniques in Pseudomyxoma peritonei. EJSO 06.552
Andreasson H, Graf W, Nygren P, Glimelius B, Mahteme H (2012) Outcome differences between debulking surgery and cytoreductive surgery in patients with Pseudomyxoma peritonei. Eur J Surg Oncol 38(10):962–968
Sugarbaker PH, Chang D (1999) Results of treatment of 385 patients with peritoneal surface spread of appendiceal malignancy. Ann Surg Oncol 6:727–731
Arjona-Sánchez A, Muñoz Casares FC, Rufián Peña S, Diaz Nieto, Casado Adam A, Rubio Perez MJ, Ortega Salas R (2011) Pseudomyxoma peritonei treated by cytoreductive surgery and hyperthermic intraperitoneal chemotherapy: results from a single centre. Clin Transl Oncol 13:261–267
Jaquet P, Sugarbaker PH (1996) Current methodologies for clinical assessment of patients with peritoneal carcinomatosis. J Exp Clin Cancer Res 15:49–58
Muñoz-Casares FC, Rufián S, Rubio MJ, Lizárraga E, Díaz-Iglesias C, Aranda E, Ciria R, Muntané J, Barrios P, Torres-Melero J, González-Moreno S, González-Bayón L, Camps B, Bretcha P, Farré J, Ortega-Pérez G, Gómez Portilla A (2007) Treatment of peritoneal carcinomatosis from ovarian cancer. Present, future directions and proposals. Clin Transl Oncol 9:652–662
Younan R, Kusamura S, Baratti D, Cloutier AS, Deraco M (2008) Morbidity, toxicity, and mortality classification systems in the local regional treatment of peritoneal surface malignancy. J Surg Oncol 98(4):253
Gough DB, Donohue JH, Schutt AJ, Gonchoroff N, Goellner JR, Wilson TO et al (1994) Pseudomyxoma peritonei. Long-term patient survival with an aggressive regional approach. Ann Surg 219:112–119
Miner TJ, Shia J, Jaques DP, Klimstra DS, Brennan MF, Coit DG (2005) Long-term survival following treatment of Pseudomyxoma peritonei: an analysis of surgical therapy. Ann Surg 241:300–308
Gonzalez-Moreno S, Sugarbaker PH (2004) Right hemicolectomy does not confer a survival advantage in patients with mucinous carcinoma of the appendix and peritoneal seeding. Br J Surg 91:304–311
Elias D, Honoré C, Ciuchendea R, Billard V, Raynard B, Lo Dico R, Dromain C, Duvillard P, Goeré D (2008) Peritoneal pseudomyxoma: results of a systematic policy of complete cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. Br J Surg 95:1164–1171
Cioppa T, Vaira M, Bing C, D’amico S, Bruscino A, de Simone M (2008) Cytoreduction and hyperthermic intraperitoneal chemotherapy in the treatment of peritoneal carcinomatosis from Pseudomyxoma peritonei. World J Gastroenterol 14:6817–6823
Yan TD, Links M, Xu ZY, Kam C, Glenn D, Morris DL (2006) Cytoreductive surgery and perioperative intraperitoneal chemotherapy for Pseudomyxoma peritonei from appendiceal mucinous neoplasms. Br J Surg 93:1270–1276
Misdraji J, Yantiss RK, Graeme-Cook FM, Balis UJ, Young RH (2003) Appendiceal mucinous neoplasm’s: a clinicopathologic analysis of 107 cases. Am J Surg Pathol 27:1089
Guo AT, Li YM, Wei LX (2012) Pseudomyxoma peritonei of 92 Chinese patients: clinical characteristics, pathological classification and prognostic factors. World J Gastroenterol 18(24):3081–3088
Elias D, Gilly F, Quenet F, Bereder JM, Sideris L, Mansvelt B, Lorimier G, Glehen O (2010) Pseudomyxoma peritonei: a French multicentric study of 301 patients treated with cytoreductive surgery and intraperitoneal chemotherapy. EJSO 36:456
Bradley RF, Stewart JH 4th, Russell GB, Levine EA, Geisinger KR (2006) Pseudomyxoma peritonei of appendiceal origin: a clinicopathologic analysis of 101 patients uniformly treated at a single institution, with literature review. Am J Surg Pathol 30(5):551–559
Carr NJ, Finch J, Ilesley IC et al (2012) Pathology and prognosis in Pseudomyxoma peritonei: a review of 274 cases. J Clin Pathol. 10.1136/jclinpath-2012-200843
Ronnet BM, Yan H, Kurman RJ, Shmookler BM, Wu L, Sugarbaker PH (2001) Patients with Pseudomyxoma peritonei associated with disseminated peritoneal adenomucinosis have a significantly more favorable prognosis than patients with peritoneal mucinous carcinomatosis. Cancer 92:85–91
Baratti D, Kusamura S, Nonaka D, Cabras AD, Laterza B, Deraco M (2009) Pseudomyxoma peritonei. Ann Surg 249:243–249
Carmignani CP, Hampton R, Sugarbaker CE, Chang D, Sugarbaker PH (2004) Utility of CEA y CA 19.9 tumors makers in diagnosis and prognostic assessment of mucinous epithelial cancers of the appendix. J Surg Oncol 87:162–166
Van de Vaart PJ, van der Vange N, Zoetmulder FA, van Goethem AR, van Tellingen O, ten Bokkel Huinink WW (1998) Intraperitoneal cisplatin with regional hyperthermia in advanced ovarían cáncer: pharmacokinetics and cisplatin-DNA adducts formation in patients. Eur J Cancer 34:148–154
Shapiro JF, Chase JL, Wolff RA et al (2010) Modern systemic chemotherapy in surgically unresectable neoplasm of appendiceal origin: a single institution experience. Cancer 116:316–322
Sugarbaker PH, Bijelic L, Chang D et al (2010) Neoadjuvant FOLFOX chemotherapy in 34 consecutive patients with mucinous peritomucinous peritoneal carcinomatosis of appendiceal origin. J Surg Oncol 102:576–581
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Arjona-Sanchez, A., Muñoz-Casares, F.C., Casado-Adam, A. et al. Outcome of Patients with Aggressive Pseudomyxoma Peritonei Treated by Cytoreductive Surgery and Intraperitoneal Chemotherapy. World J Surg 37, 1263–1270 (2013). https://doi.org/10.1007/s00268-013-2000-2
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00268-013-2000-2