Background: The current treatment for pseudomyxoma peritonei (PMP) consists of radical cytoreductive surgery (CRS) followed by hyperthermic intra-peritoneal chemotherapy (HIPEC). Aim: To assess PMP patients regarding the clinical and pathological characteristics, the treatment including surgery (CRS) and chemotherapy either HIPEC type or post-operative systemic chemotherapy aiming to evaluate end results regarding recurrence and survival. Patients and Methods: This retrospective study included 39 patients with PMP who were diagnosed, treated and followed-up from 2009-2014 at National Cancer Institute, Cairo, Egypt. Results: High grade mucinous adenocarcinoma was found in 23.1% of patients. Patients with low grade tumor showed higher survival rate compared with patients with high grade disease. The mean operative PCI score (peritoneal cancer index) that was done to all patients who were explored was 15.81. Our study reported success to achieve complete cytoreduction that was combined with HIPEC in 44% of patients who were planned for this modality. Treatment related postoperative grade (3 - 5) complications mainly surgery related developed in 17.3 of patients. Operative mortality was 22.2%. The follow up period in our study was quite short (mean 22.9). However the overall survival at the end of the follow up in our study was 48.7%, 1 year survival was 82%, and 2 year survival was 41%. The overall survival in patients treated with CRS and HIPEC was 66.6%, with 1 year and 2 year survival of 91% and 66.6% respectively. Only 2 patients developed recurrent disease during the follow up period. Conclusions: The outcome of PMP treatment process is extremely variable. Combined CRS and HIPEC is considered the best therapeutic approach for patients with PMP. Surgical experience combined with proper patient selection have to be built up together to improve the outcome. That could only be achieved through more centralization of patients’ treatment in specialized units or center.
KeywordsHIPECNCI ExperiencePseudomyxoma Peritonii
Andreasson, H., Lorant, T., Pahlman, L., Graf, W. and Mahteme, H. (2014) Cytoreductive Surgery plus Perioperative Intraperitoneal Chemotherapy in Pseudomyxoma Peritonei: Aspects of the Learning Curve. European Journal of Surgical Oncology, 40, 930-936. https://doi.org/10.1016/j.ejso.2014.03.001
McBride, K., McFadden, D. and Osler, T. (2013) Improved Survival of Patients with Pseudomyxoma Peritonei Receiving Intraperitoneal Chemotherapy with Cytoreductive Surgery: A Systematic Review and Meta-Analysis. Journal of Surgical Research, 183, 246-252. https://doi.org/10.1016/j.jss.2012.12.040
Alves, S., Mohamed, F., Yadegarfar, G., Youssef, H. and Moran, B.J. (2010) Prospective Longitudinal Study of Quality of Life Following Cytoreductive Surgery and Intraperitoneal Chemotherapy for Pseudomyxoma Peritonei. European Journal of Surgical Oncology, 36, 1156-1161. https://doi.org/10.1016/j.ejso.2010.09.004
Chua, T.C., Yan, T.D., Smigielski, M.E., Zhu, K.J., Ng, K.M., Zhao, J. and Morris, D.L. (2009) Long-Term Survival in Patients with Pseudomyxoma Peritonei Treated with Cytoreductive Surgery and Perioperative Intraperitoneal Chemotherapy: 10 Years of Experience from a Single Institution. Annals of Surgical Oncology, 16, 1903-1911. https://doi.org/10.1245/s10434-009-0341-8
Lord, A.C., Shihab, O., Chandrakumaran, K., Mohamed, F., Cecil, T.D. and Moran, B.J. (2014) Recurrence and Outcome after Complete Tumour Removal and Hyperthermic Intraperitoneal Chemotherapy in 512 Patients with Pseudomyxoma Peritonei from Perforated Appendiceal Mucinous Tumors. European Journal of Surgical Oncology, 41, 396-399. https://doi.org/10.1016/j.ejso.2014.08.476
Smeenk, R.M., Verwaal, V.J., Antonini, N. and Zoetmulder, F.A. (2007) Survival Analysis of Pseudomyxoma Peritonei Patients Treated by Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy. Annals of Surgery, 245, 104-109. https://doi.org/10.1097/01.sla.0000231705.40081.1a
Chua, T.C., Moran, B.J., Sugarbaker, P.H., Levine, E.A., Glehen, O., Gilly, F.N., Baratti, D., Deraco, M., Elias, D., Sardi, A., Liauw, W., Yan, T.D., Barrios, P., Gómez Portilla, A., de Hingh, I.H., Ceelen, W.P., Pelz, J.O., Piso, P., González-Moreno, S., Van Der Speeten, K. and Morris, D.L. (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. Journal of Clinical Oncology, 30, 2449-2456. https://doi.org/10.1200/JCO.2011.39.7166
Elias, D., Gilly, F., Boutitie, F., Quenet, F., Bereder, J.M., Mansvelt, B., Lorimier, G., Dubè, P. and Glehen, O. (2010) Peritoneal Colorectal Carcinomatosis Treated with Surgery and Perioperative Intraperitoneal Chemotherapy: Retrospective Analysis of 523 Patients from a Multicentric French Study. Journal of Clinical Oncology, 28, 63-68. https://doi.org/10.1200/JCO.2009.23.9285
Taflampas, P., Dayal, S., Chandrakumaran, K., Mohamed, F., Cecil, T.D. and Moran, B.J. (2014) Pre-Operative Tumour Marker Status Predicts Recurrence and Survival after Complete Cytoreduction and Hyperthermic Intraperitoneal Chemotherapy for Appendiceal Pseudomyxoma Peritonei: Analysis of 519 Patients. European Journal of Surgical Oncology, 40, 515-520. https://doi.org/10.1016/j.ejso.2013.12.021
Panarelli, N.C. and Yantiss, R.K. (2011) Mucinous Neoplasms of the Appendix and Peritoneum. Archives of Pathology & Laboratory Medicine, 135, 1261-1268. https://doi.org/10.5858/arpa.2011-0034-RA
Sugarbaker, P.H. (2005) Cytoreductive Surgery and Perioperative Intraperitoneal Chemotherapy: A New Standard of Care for Appendiceal Mucinous Tumors with Peritoneal Dissemination. Clinics in Colon and Rectal Surgery, 18, 204-214. https://doi.org/10.1055/s-2005-916281
Sugarbaker, P.H. (2013) Pseudomyxoma Peritonei and Peritoneal Metastases from Appendiceal Malignancy. In: Sugarbaker, P.H., Ed., Cytoreductive Surgery and Perioperative Chemotherapy for Peritoneal Surface Malignancy. Textbook and Video Atlas, 3, 57-75.
Sugarbaker, P.H. and Esquivel, J. (2000) Clinical Presentation of the Pseudomyxoma Peritonei Syndrome. British Journal of Surgery, 87, 1414-1418. https://doi.org/10.1046/j.1365-2168.2000.01553.x
de Bree, E., Koops, W., Kroger, R., van Ruth, S., Verwaal, V.J. and Zoetmulder, F.A. (2006) Preoperative Computed Tomography and Selection of Patients with Colorectal Peritoneal Carcinomatosis for Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy. European Journal of Surgical Oncology, 32, 65-71. https://doi.org/10.1016/j.ejso.2005.09.016
Menassel, B., Duclos, A., Passot, G., Dohan, A., Payet, C., Isaac, S., Valette, P.J., Glehen, O. and Rousset, P. (2016) Preoperative CT and MRI Prediction of Non-Resectability in Patients Treated for Pseudomyxoma Peritonei from Mucinous Appendiceal Neoplasms. European Journal of Surgical Oncology, 42, 558-566. https://doi.org/10.1016/j.ejso.2016.01.005
Elias, D., Lefevre, J.H., Chevalier, J., Brouquet, A., Marchal, F., Classe, J.M., Ferron, G., Guilloit, J.M., Meeus, P., Goéré, D. and Bonastre, J. (2009) Complete Cytoreductive Surgery plus Intraperitoneal Chemohyperthermia with Oxaliplatin for Peritoneal Carcinomatosis of Colorectal Origin. Journal of Clinical Oncology, 27, 681-685. https://doi.org/10.1200/JCO.2008.19.7160
Jarvinen, P., Ristimaki, A., Kantonen, J. and Lepisto, A. (2013) Feasibility of Radical Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy for Pseudomyxoma Peritonei of Appendicular Origin. Scandinavian Journal of Surgery, 102, 145-151. https://doi.org/10.1177/1457496913490463
Andreasson, H., Graf, W., Nygren, P., Glimelius, B. and Mahteme, H. (2012) Outcome Differences between Debulking Surgery and Cytoreductive Surgery in Patients with Pseudomyxoma Peritonei. European Journal of Surgical Oncology, 38, 962-968. https://doi.org/10.1016/j.ejso.2012.07.009
Harmon, R.L. and Sugarbaker, P.H. (2005) Prognostic Indicators in Peritoneal Carcinomatosis from Gastrointestinal Cancer. International Seminars in Surgical Oncology, 2, 3. https://doi.org/10.1186/1477-7800-2-3
Sugarbaker, P. and Chang, D. (1999) Results of Treatment of 385 Patients with Peritoneal Surface Spread of Appendiceal Malignancy. Annals of Surgical Oncology, 6, 727-731. https://doi.org/10.1007/s10434-999-0727-7
Sugarbaker, P.H., Bijelic, L., Chang, D. and Yoo, D. (2010) Neoadjuvant FOLFOX Chemotherapy in 34 Consecutive Patients with Mucinous Peritoneal Carcinomatosis of Appendiceal Origin. Journal of Surgical Oncology, 102, 576-581. https://doi.org/10.1002/jso.21679
Chen, C.-F., Huang, C.-J., Kang, W.-Y. and Hsieh, J.-S. (2008) Experience with Adjuvant Chemotherapy for Pseudomyxoma Peritonei Secondary to Mucinous Adenocarcinoma of the Appendix with Oxaliplatin/Fluorouracil/Leucovorin (FOLFOX4). World Journal of Surgical Oncology, 6, 118. https://doi.org/10.1186/1477-7819-6-118
Yan, T.D., Black, D., Savady, R. and Sugarbaker, P.H. (2007) A Systematic Review on the Efficacy of Cytoreductive Surgery and Perioperative Intraperitoneal Chemotherapy for Pseudomyxoma Peritonei. Annals of Surgical Oncology, 14, 484-492. https://doi.org/10.1245/s10434-006-9182-x
Stephens, A.D., Alderman, R., Chang, D., Edwards, G.D., Esquivel, J., Sebbag, G., Steves, M.A. and Sugarbaker, P.H. (1999) Morbidity and Mortality of 200 Treatments with Cytoreductive Surgery and Hyperthermic Intraoperative Intraperitoneal Chemotherapy Using the Coliseum Technique. Annals of Surgical Oncology, 6, 790-796. https://doi.org/10.1007/s10434-999-0790-0
Smeenk, R.M., Verwaal, V.J. and Zoetmulder, F.A.N. (2006) Toxicity and Mortality of Cytoreduction and Intraoperative Hyperthermic Intraperitoneal Chemotherapy in Pseudomyxoma Peritonei—A Report of 103 Procedures. European Journal of Surgical Oncology, 32, 186-190. https://doi.org/10.1016/j.ejso.2005.08.009
Baratti, D., Kusamura, S., Nonaka, D., Langer, M., Andreola, S., Favaro, M., Gavazzi, C., Laterza, B. and Deraco, M. (2008) Pseudomyxoma Peritonei: Clinical Pathological and Biological Prognostic Factors in Patients Treated with Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy (HIPEC). Annals of Surgical Oncology, 15, 526-534. https://doi.org/10.1245/s10434-007-9691-2
Mohr, Z., Hirche, C., Liebeskind, U., Rau, B.M. and Hünerbein, M. (2011) Feasibility of Delayed Hyperthermic Intraperitoneal Chemotherapy in Case of Unforeseen Complications. European Surgical Research, 47, 19-25. https://doi.org/10.1159/000327397
Youssef, H., Newman, C., Chandrakumaran, K., Mohamed, F., Cecil, T.D. and Moran, B.J. (2011) Operative Findings, Early Complications, and Long-Term Survival in 456 Patients with Pseudomyxoma Peritonei Syndrome of Appendiceal Origin. Diseases of the Colon & Rectum, 54, 293-299. https://doi.org/10.1007/DCR.0b013e318202f026
Kusamura, S., Moran, B.J., Sugarbaker, P.H., Levine, E.A., Elias, D., Baratti, D., Morris, D.L., Sardi, A., Glehen, O., Deraco, M., Peritoneal Surface Oncology Group International (PSOGI), et al. (2014) Multicentre Study of the Learning Curve and Surgical Performance of Cytoreductive Surgery with Intraperitoneal Chemotherapy for Pseudomyxoma Peritonei. British Journal of Surgery, 101, 1758-1765. https://doi.org/10.1002/bjs.9674
Krishnamurthy, S. and Balasubramaniam, R. (2016) Role of Imaging in Peritoneal Surface Malignancies. Indian Journal of Surgical Oncology, 7, 441-452. https://doi.org/10.1007/s13193-016-0539-8