{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,5]],"date-time":"2026-11-05T04:42:35Z","timestamp":1793853755489,"version":"4.6.1"},"reference-count":67,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2021,2,13]],"date-time":"2021-02-13T00:00:00Z","timestamp":1613174400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2021,2,13]],"date-time":"2021-02-13T00:00:00Z","timestamp":1613174400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100009601","name":"Beijing Municipal Administration of Hospitals","doi-asserted-by":"publisher","award":["DFL20180701"],"award-info":[{"award-number":["DFL20180701"]}],"id":[{"id":"10.13039\/501100009601","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100009601","name":"Beijing Municipal Administration of Hospitals","doi-asserted-by":"publisher","award":["2017400003235J007"],"award-info":[{"award-number":["2017400003235J007"]}],"id":[{"id":"10.13039\/501100009601","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Beijing Municipal Science & Technology Comission","award":["Z161100000516077"],"award-info":[{"award-number":["Z161100000516077"]}]},{"name":"Beijing Shijitan Hospital, Capital Medical University","award":["2016fmzlwk"],"award-info":[{"award-number":["2016fmzlwk"]}]},{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["82073376"],"award-info":[{"award-number":["82073376"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Orphanet J Rare Dis"],"published-print":{"date-parts":[[2021,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>Pseudomyxoma peritonei (PMP) is a clinical malignant syndrome mainly originating from the appendix, with an incidence of 2\u20134 per million people. As a rare disease, an early and accurate diagnosis of PMP is difficult. It was not until the 1980s that the systematic study of this disease was started.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Main body<\/jats:title>\n                <jats:p>As a result of clinical and basic research progress over the last 4 decades, a comprehensive strategy based on cytoreductive surgery (CRS)\u2009+\u2009hyperthermic intraperitoneal chemotherapy (HIPEC) has been established and proved to be an effective treatment for PMP. Currently, CRS\u2009+\u2009HIPEC was recommended as the standard treatment for PMP worldwide. There are several consensuses on PMP management, playing an important role in the standardization of CRS\u2009+\u2009HIPEC. However, controversies exist among consensuses published worldwide. A systematic evaluation of PMP consensuses helps not only to standardize PMP treatment but also to identify existing controversies and point to possible solutions in the future. The controversy underlying the consensus and vice versa promotes the continuous refinement and updating of consensuses and continue to improve PMP management through a gradual and continuous process. In this traditional narrative review, we systemically evaluated the consensuses published by major national and international academic organizations, aiming to get a timely update on the treatment strategies of CRS\u2009+\u2009HIPEC on PMP.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusion<\/jats:title>\n                <jats:p>Currently, consensuses have been reached on the following aspects: pathological classification, terminology, preoperative evaluation, eligibility for surgical treatment, maximal tumor debulking, CRS technical details, and severe adverse event classification system. However, controversies still exist regarding the HIPEC regimen, systemic chemotherapy, and\u00a0early postoperative intraperitoneal chemotherapy.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s13023-021-01723-6","type":"journal-article","created":{"date-parts":[[2021,2,13]],"date-time":"2021-02-13T20:41:30Z","timestamp":1613248890000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":62,"title":["Consensuses and controversies on pseudomyxoma peritonei: a review of the published consensus statements and guidelines"],"prefix":"10.1186","volume":"16","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8557-7495","authenticated-orcid":false,"given":"Yu-Lin","family":"Lin","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4696-1881","authenticated-orcid":false,"given":"Da-Zhao","family":"Xu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1253-8361","authenticated-orcid":false,"given":"Xin-Bao","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8815-1692","authenticated-orcid":false,"given":"Feng-Cai","family":"Yan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hong-Bin","family":"Xu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zheng","family":"Peng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6018-6538","authenticated-orcid":false,"given":"Yan","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,2,13]]},"reference":[{"key":"1723_CR1","doi-asserted-by":"publisher","first-page":"196","DOI":"10.1016\/j.ejso.2007.04.002","volume":"34","author":"RM Smeenk","year":"2008","unstructured":"Smeenk RM, van Velthuysen ML, Verwaal VJ, Zoetmulder FA. Appendiceal neoplasms and pseudomyxoma peritonei: a population based study. Eur J Surg Oncol. 2008;34:196\u2013201.","journal-title":"Eur J Surg Oncol"},{"key":"1723_CR2","doi-asserted-by":"publisher","first-page":"511","DOI":"10.1080\/02656736.2017.1310938","volume":"33","author":"R Mittal","year":"2017","unstructured":"Mittal R, Chandramohan A, Moran B. Pseudomyxoma peritonei: natural history and treatment. Int J Hyperthermia. 2017;33:511\u20139.","journal-title":"Int J Hyperthermia"},{"key":"1723_CR3","first-page":"256","volume":"40","author":"JS Spratt","year":"1980","unstructured":"Spratt JS, Adcock RA, Muskovin M, Sherrill W, McKeown J. Clinical delivery system for intraperitoneal hyperthermic chemotherapy. Cancer Res. 1980;40:256\u201360.","journal-title":"Cancer Res"},{"key":"1723_CR4","unstructured":"Reviews: from systematic to narrative: narrative review. https:\/\/guides.library.uab.edu\/c.php?g=63689&p=409774."},{"key":"1723_CR5","unstructured":"Narrative review. https:\/\/www.sciencedirect.com\/topics\/psychology\/narrative-review."},{"key":"1723_CR6","doi-asserted-by":"publisher","first-page":"2525","DOI":"10.1002\/cncr.32881","volume":"126","author":"Chicago Consensus Working Group","year":"2020","unstructured":"Chicago Consensus Working Group. The Chicago consensus on peritoneal surface malignancies: management of appendiceal neoplasms. Cancer. 2020;126:2525\u201333.","journal-title":"Cancer"},{"key":"1723_CR7","first-page":"1527","volume":"99","author":"Y Li","year":"2019","unstructured":"Li Y, Xu HB, Peng Z, Cui SZ, Wu W. Chinese expert consensus on cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for pseudomyxoma peritonei. Natl Med J China. 2019;99:1527\u201335.","journal-title":"Natl Med J China"},{"key":"1723_CR8","doi-asserted-by":"publisher","first-page":"1800","DOI":"10.1016\/j.ejso.2018.06.029","volume":"44","author":"Latin American Registry of Peritoneal Diseases","year":"2018","unstructured":"Latin American Registry of Peritoneal Diseases. Current practice of Latin American centers in the treatment of peritoneal diseases with cytoreductive surgery with HIPEC. Eur J Surg Oncol. 2018;44:1800\u20134.","journal-title":"Eur J Surg Oncol"},{"key":"1723_CR9","doi-asserted-by":"publisher","first-page":"530","DOI":"10.1590\/0100-69912017005016","volume":"44","author":"TP Batista","year":"2017","unstructured":"Batista TP, Sarmento BJQ, Loureiro JF, Petruzziello A, Lopes A, Santos CC, et al. A proposal of Brazilian Society of Surgical Oncology (BSSO\/SBCO) for standardizing cytoreductive surgery (CRS) plus hyperthermic intraperitoneal chemotherapy (HIPEC) procedures in Brazil: pseudomixoma peritonei, appendiceal tumors and malignant peritoneal mesothelioma. Revista do Colegio Brasileiro de Cirurgioes. 2017;44:530\u201344.","journal-title":"Revista do Colegio Brasileiro de Cirurgioes"},{"key":"1723_CR10","doi-asserted-by":"publisher","first-page":"160","DOI":"10.1007\/s13193-016-0500-x","volume":"7","author":"A Bhatt","year":"2016","unstructured":"Bhatt A, Mehta S, Seshadri RA, Sethna K, Zaveri S, Rajan F, et al. The initial Indian experience with cytoreductive surgery and HIPEC in the treatment of peritoneal metastases. Indian J Surg Oncol. 2016;7:160\u20135.","journal-title":"Indian J Surg Oncol"},{"key":"1723_CR11","doi-asserted-by":"publisher","first-page":"1390","DOI":"10.1097\/00000478-199512000-00006","volume":"19","author":"BM Ronnett","year":"1995","unstructured":"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 \u201cpseudomyxoma peritonei.\u201d Am J Surg Pathol. 1995;19:1390\u2013408.","journal-title":"Am J Surg Pathol."},{"key":"1723_CR12","doi-asserted-by":"publisher","first-page":"551","DOI":"10.1097\/01.pas.0000202039.74837.7d","volume":"30","author":"RF Bradley","year":"2006","unstructured":"Bradley RF, Stewart JH, Russell GB, Levine EA, Geisinger KR. Pseudomyxoma peritonei of appendiceal origin: a clinicopathologic analysis of 101 patients uniformly treated at a single institution, with literature review. Am J Surg Pathol. 2006;30:551\u20139.","journal-title":"Am J Surg Pathol"},{"key":"1723_CR13","first-page":"122","volume-title":"WHO classification of tumours of the digestive system","author":"N Carr","year":"2010","unstructured":"Carr N, Sobin L. Adenocarcinoma of the appendix. In: Bosman F, Carneiro C, Hruban R, Theise N, editors. WHO classification of tumours of the digestive system. Lyon: IARC Press; 2010. p. 122\u20135."},{"key":"1723_CR14","doi-asserted-by":"publisher","first-page":"277","DOI":"10.1002\/jso.21054","volume":"98","author":"B Moran","year":"2008","unstructured":"Moran B, Baratti D, Yan TD, Kusamura S, Deraco M. Consensus statement on the loco-regional treatment of appendiceal mucinous neoplasms with peritoneal dissemination (pseudomyxoma peritonei). J Surg Oncol. 2008;98:277\u201382.","journal-title":"J Surg Oncol"},{"key":"1723_CR15","doi-asserted-by":"publisher","first-page":"14","DOI":"10.1097\/PAS.0000000000000535","volume":"40","author":"NJ Carr","year":"2016","unstructured":"Carr NJ, Cecil TD, Mohamed F, Sobin LH, Sugarbaker PH, Gonzalez-Moreno S, et al. A consensus for classification and pathologic reporting of pseudomyxoma peritonei and associated appendiceal neoplasia: the results of the Peritoneal Surface Oncology Group International (PSOGI) modified Delphi process. Am J Surg Pathol. 2016;40:14\u201326.","journal-title":"Am J Surg Pathol"},{"key":"1723_CR16","doi-asserted-by":"publisher","first-page":"38","DOI":"10.1097\/PAP.0000000000000178","volume":"25","author":"MA Valasek","year":"2018","unstructured":"Valasek MA, Pai RK. An update on the diagnosis, grading, and staging of appendiceal mucinous neoplasms. Adv Anat Pathol. 2018;25:38\u201360.","journal-title":"Adv Anat Pathol"},{"key":"1723_CR17","doi-asserted-by":"publisher","first-page":"404","DOI":"10.1245\/s10434-017-6252-1","volume":"25","author":"D Baratti","year":"2018","unstructured":"Baratti D, Kusamura S, Milione M, Bruno F, Guaglio M, Deraco M. Validation of the recent PSOGI pathological classification of pseudomyxoma peritonei in a single-center series of 265 patients treated by cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. Ann Surg Oncol. 2018;25:404\u201313.","journal-title":"Ann Surg Oncol"},{"key":"1723_CR18","doi-asserted-by":"publisher","first-page":"228","DOI":"10.1002\/jso.21068","volume":"98","author":"AG Portilla","year":"2008","unstructured":"Portilla AG, Shigeki K, Dario B, Marcello D. The intraoperative staging systems in the management of peritoneal surface malignancy. J Surg Oncol. 2008;98:228\u201331.","journal-title":"J Surg Oncol"},{"key":"1723_CR19","doi-asserted-by":"publisher","first-page":"2449","DOI":"10.1200\/JCO.2011.39.7166","volume":"30","author":"TC Chua","year":"2012","unstructured":"Chua TC, Moran BJ, Sugarbaker PH, Levine EA, Glehen O, Gilly FN, et al. 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. 2012;30:2449\u201356.","journal-title":"J Clin Oncol"},{"key":"1723_CR20","doi-asserted-by":"publisher","first-page":"341","DOI":"10.1016\/j.ygyno.2013.08.005","volume":"131","author":"A Fagotti","year":"2013","unstructured":"Fagotti A, Vizzielli G, Fanfani F, Costantini B, Ferrandina G, Gallotta V, et al. Introduction of staging laparoscopy in the management of advanced epithelial ovarian, tubal and peritoneal cancer: impact on prognosis in a single institution experience. Gynecol Oncol. 2013;131:341\u20136.","journal-title":"Gynecol Oncol"},{"key":"1723_CR21","doi-asserted-by":"publisher","first-page":"29","DOI":"10.1097\/00000658-199501000-00004","volume":"221","author":"PH Sugarbaker","year":"1995","unstructured":"Sugarbaker PH. Peritonectomy procedures. Ann Surg. 1995;221:29\u201342.","journal-title":"Ann Surg"},{"key":"1723_CR22","doi-asserted-by":"publisher","first-page":"703","DOI":"10.1016\/S1055-3207(03)00048-6","volume":"12","author":"PH Sugarbaker","year":"2003","unstructured":"Sugarbaker PH. Peritonectomy procedures. Surg Oncol Clin N Am. 2003;12:703\u201327.","journal-title":"Surg Oncol Clin N Am."},{"key":"1723_CR23","doi-asserted-by":"publisher","first-page":"635","DOI":"10.1245\/ASO.2006.03.079","volume":"13","author":"PH Sugarbaker","year":"2006","unstructured":"Sugarbaker PH, Alderman R, Edwards G, Marquardt CE, Gushchin V, Esquivel J, et al. Prospective morbidity and mortality assessment of cytoreductive surgery plus perioperative intraperitoneal chemotherapy to treat peritoneal dissemination of appendiceal mucinous malignancy. Ann Surg Oncol. 2006;13:635\u201344.","journal-title":"Ann Surg Oncol"},{"key":"1723_CR24","first-page":"210","volume-title":"Cytoreductive surgery & perioperative chemotherapy for peritoneal surface malignancy: textbook and video atlas","author":"PH Sugarbaker","year":"2017","unstructured":"Sugarbaker PH, Van der Speeten K, Stuart OA, Chang D, Mahteme H. Patient- and treatment-related variables, adverse events and their statistical relationship for treatment of peritoneal metastases. In: Sugarbaker PH, editor. Cytoreductive surgery & perioperative chemotherapy for peritoneal surface malignancy: textbook and video atlas. Canada: Cin\u00e9-Med; 2017. p. 210\u20131."},{"key":"1723_CR25","volume-title":"Cytoreductive surgery & perioperative chemotherapy for peritoneal surface malignancy: textbook and video atlas, 2","author":"N Bakrin","year":"2017","unstructured":"Bakrin N, Deraco M, Glehen O, Morris DL, Van der Speeten K. Cytoreductive surgery & perioperative chemotherapy for peritoneal surface malignancy: textbook and video atlas, 2. Canada: Cin\u00e9-Med; 2017."},{"key":"1723_CR26","doi-asserted-by":"publisher","first-page":"346","DOI":"10.1159\/000066229","volume":"63","author":"D Elias","year":"2002","unstructured":"Elias D, El Otmany A, Bonnay M, Paci A, Ducreux M, Antoun S, et al. Human pharmacokinetic study of heated intraperitoneal oxaliplatin in increasingly hypotonic solutions after complete resection of peritoneal carcinomatosis. Oncology. 2002;63:346\u201352.","journal-title":"Oncology"},{"key":"1723_CR27","doi-asserted-by":"publisher","first-page":"589","DOI":"10.1016\/j.ejso.2010.04.005","volume":"36","author":"C Pomel","year":"2010","unstructured":"Pomel C, Ferron G, Lorimier G, Rey A, Lhomme C, Classe JM, et al. Hyperthermic intra-peritoneal chemotherapy using oxaliplatin as consolidation therapy for advanced epithelial ovarian carcinoma. Results of a phase II prospective multicentre trial. CHIPOVAC study. Eur J Surg Oncol. 2010;36:589\u201393.","journal-title":"Eur J Surg Oncol."},{"key":"1723_CR28","doi-asserted-by":"publisher","first-page":"571","DOI":"10.1007\/s00280-014-2525-6","volume":"74","author":"Q Chalret du Rieu","year":"2014","unstructured":"Chalret du Rieu Q, White-Koning M, Picaud L, Lochon I, Marsili S, Gladieff L, et al. Population pharmacokinetics of peritoneal, plasma ultrafiltrated and protein-bound oxaliplatin concentrations in patients with disseminated peritoneal cancer after intraperitoneal hyperthermic chemoperfusion of oxaliplatin following cytoreductive surgery: correlation between oxaliplatin exposure and thrombocytopenia. Cancer Chemother Pharmacol. 2014;74:571\u201382.","journal-title":"Cancer Chemother Pharmacol."},{"key":"1723_CR29","doi-asserted-by":"publisher","first-page":"2315","DOI":"10.1245\/s10434-016-5143-1","volume":"23","author":"T Charrier","year":"2016","unstructured":"Charrier T, Passot G, Peron J, Maurice C, Gocevska S, Qu\u00e9net F, et al. Cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy with oxaliplatin increases the risk of postoperative hemorrhagic complications: analysis of predictive factors. Ann Surg Oncol. 2016;23:2315\u201322.","journal-title":"Ann Surg Oncol"},{"key":"1723_CR30","doi-asserted-by":"publisher","first-page":"e84","DOI":"10.3747\/co.v18i2.663","volume":"18","author":"F Mohamed","year":"2011","unstructured":"Mohamed F, Cecil T, Moran B, Sugarbaker P. A new standard of care for the management of peritoneal surface malignancy. Curr Oncol. 2011;18:e84-96.","journal-title":"Curr Oncol"},{"key":"1723_CR31","doi-asserted-by":"publisher","first-page":"573","DOI":"10.1016\/j.jamcollsurg.2013.12.013","volume":"218","author":"EA Levine","year":"2014","unstructured":"Levine EA, Stewart JH, Shen P, Russell GB, Loggie BL, Votanopoulos KI. Intraperitoneal chemotherapy for peritoneal surface malignancy: experience with 1,000 patients. J Am Coll Surg. 2014;218:573\u201385.","journal-title":"J Am Coll Surg"},{"key":"1723_CR32","doi-asserted-by":"publisher","first-page":"147","DOI":"10.1007\/s00280-010-1460-4","volume":"68","author":"K Van der Speeten","year":"2011","unstructured":"Van der Speeten K, Stuart OA, Chang D, Mahteme H, Sugarbaker PH. Changes induced by surgical and clinical factors in the pharmacology of intraperitoneal mitomycin C in 145 patients with peritoneal carcinomatosis. Cancer Chemother Pharmacol. 2011;68:147\u201356.","journal-title":"Cancer Chemother Pharmacol"},{"key":"1723_CR33","doi-asserted-by":"publisher","first-page":"1501","DOI":"10.1245\/s10434-013-3061-z","volume":"21","author":"K Turaga","year":"2014","unstructured":"Turaga K, Levine E, Barone R, Sticca R, Petrelli N, Lambert L, et al. Consensus guidelines from The American Society of Peritoneal Surface Malignancies on standardizing the delivery of hyperthermic intraperitoneal chemotherapy (HIPEC) in colorectal cancer patients in the United States. Ann Surg Oncol. 2014;21:1501\u20135.","journal-title":"Ann Surg Oncol"},{"key":"1723_CR34","doi-asserted-by":"publisher","first-page":"1903","DOI":"10.1245\/s10434-009-0341-8","volume":"16","author":"TC Chua","year":"2009","unstructured":"Chua TC, Yan TD, Smigielski ME, Zhu KJ, Ng KM, Zhao J, et al. Long-term survival in patients with pseudomyxoma peritonei treated with cytoreductive surgery and perioperative intraperitoneal chemotherapy: 10 years of experience from a single institution. Ann Surg Oncol. 2009;16:1903\u201311.","journal-title":"Ann Surg Oncol"},{"key":"1723_CR35","doi-asserted-by":"publisher","first-page":"456","DOI":"10.1016\/j.ejso.2010.01.006","volume":"36","author":"D Elias","year":"2010","unstructured":"Elias D, Gilly F, Quenet F, Bereder JM, Sideris L, Mansvelt B, et al. Pseudomyxoma peritonei: a French multicentric study of 301 patients treated with cytoreductive surgery and intraperitoneal chemotherapy. Eur J Surg Oncol. 2010;36:456\u201362.","journal-title":"Eur J Surg Oncol"},{"key":"1723_CR36","doi-asserted-by":"publisher","first-page":"1291","DOI":"10.1245\/s10434-009-0875-9","volume":"17","author":"A Saxena","year":"2010","unstructured":"Saxena A, Yan TD, Chua TC, Morris DL. Critical assessment of risk factors for complications after cytoreductive surgery and perioperative intraperitoneal chemotherapy for pseudomyxoma peritonei. Ann Surg Oncol. 2010;17:1291\u2013301.","journal-title":"Ann Surg Oncol"},{"key":"1723_CR37","doi-asserted-by":"publisher","first-page":"769","DOI":"10.1097\/SLA.0b013e31820b4dba","volume":"253","author":"TC Chua","year":"2011","unstructured":"Chua TC, Liauw W, Zhao J, Morris DL. Upfront compared to delayed cytoreductive surgery and perioperative intraperitoneal chemotherapy for pseudomyxoma peritonei is associated with considerably lower perioperative morbidity and recurrence rate. Ann Surg. 2011;253:769\u201373.","journal-title":"Ann Surg"},{"key":"1723_CR38","doi-asserted-by":"publisher","first-page":"293","DOI":"10.1007\/DCR.0b013e318202f026","volume":"54","author":"H Youssef","year":"2011","unstructured":"Youssef H, Newman C, Chandrakumaran K, Mohamed F, Cecil TD, Moran BJ. Operative findings, early complications, and long-term survival in 456 patients with pseudomyxoma peritonei syndrome of appendiceal origin. Dis Colon Rectum. 2011;54:293\u20139.","journal-title":"Dis Colon Rectum"},{"key":"1723_CR39","doi-asserted-by":"publisher","first-page":"1366","DOI":"10.1097\/DCR.0b013e3182a62b0d","volume":"56","author":"S Dayal","year":"2013","unstructured":"Dayal S, Taflampas P, Riss S, Chandrakumaran K, Cecil TD, Mohamed F, et al. Complete cytoreduction for pseudomyxoma peritonei is optimal but maximal tumor debulking may be beneficial in patients in whom complete tumor removal cannot be achieved. Dis Colon Rectum. 2013;56:1366\u201372.","journal-title":"Dis Colon Rectum"},{"key":"1723_CR40","first-page":"569","volume":"68","author":"M Robella","year":"2013","unstructured":"Robella M, Vaira M, Marsanic P, Mellano A, Cinquegrana A, Sottile A, et al. Treatment of pseudomyxoma peritonei with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC): a single center experience. Minerva Chir. 2013;68:569\u201377.","journal-title":"Minerva Chir"},{"key":"1723_CR41","doi-asserted-by":"publisher","first-page":"21","DOI":"10.9738\/INTSURG-D-14-00089.1","volume":"100","author":"DS Sparks","year":"2015","unstructured":"Sparks DS, Morris B, Xu W, Fulton J, Atkinson V, Meade B, et al. Cytoreductive surgery and heated intraperitoneal chemotherapy for peritoneal carcinomatosis secondary to mucinous adenocarcinoma of the appendix. Int Surg. 2015;100:21\u20138.","journal-title":"Int Surg"},{"key":"1723_CR42","doi-asserted-by":"publisher","first-page":"1035","DOI":"10.1016\/j.ejso.2016.03.017","volume":"42","author":"N Ansari","year":"2016","unstructured":"Ansari N, Chandrakumaran K, Dayal S, Mohamed F, Cecil TD, Moran BJ. Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy in 1000 patients with perforated appendiceal epithelial tumours. Eur J Surg Oncol. 2016;42:1035\u201341.","journal-title":"Eur J Surg Oncol"},{"key":"1723_CR43","doi-asserted-by":"publisher","first-page":"452","DOI":"10.1007\/s12664-017-0799-4","volume":"36","author":"AZ Azzam","year":"2017","unstructured":"Azzam AZ, Alyahya ZA, Wusaibie AAA, Amin TM. Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy in the management of pseudomyxoma peritonei: a single-center experience. Indian J Gastroenterol. 2017;36:452\u20138.","journal-title":"Indian J Gastroenterol"},{"key":"1723_CR44","doi-asserted-by":"publisher","first-page":"354","DOI":"10.4251\/wjgo.v9.i9.354","volume":"9","author":"TE Grotz","year":"2017","unstructured":"Grotz TE, Royal RE, Mansfield PF, Overman MJ, Mann GN, Robinson KA, et al. Stratification of outcomes for mucinous appendiceal adenocarcinoma with peritoneal metastasis by histological grade. World J Gastrointest Oncol. 2017;9:354\u201362.","journal-title":"World J Gastrointest Oncol"},{"key":"1723_CR45","doi-asserted-by":"publisher","first-page":"76","DOI":"10.1245\/s10434-017-6214-7","volume":"25","author":"HA Choudry","year":"2018","unstructured":"Choudry HA, Pai RK, Shuai Y, Ramalingam L, Jones HL, Pingpank JF, et al. Impact of cellularity on oncologic outcomes following cytoreductive surgery and hyperthermic intraperitoneal chemoperfusion for pseudomyxoma peritonei. Ann Surg Oncol. 2018;25:76\u201382.","journal-title":"Ann Surg Oncol"},{"key":"1723_CR46","doi-asserted-by":"publisher","first-page":"668","DOI":"10.1002\/bjs.10716","volume":"105","author":"JB Delhorme","year":"2018","unstructured":"Delhorme JB, Severac F, Averous G, Glehen O, Passot G, Bakrin N, et al. Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for pseudomyxoma peritonei of appendicular and extra-appendicular origin. Br J Surg. 2018;105:668\u201376.","journal-title":"Br J Surg"},{"key":"1723_CR47","doi-asserted-by":"publisher","first-page":"166","DOI":"10.1007\/s13193-015-0478-9","volume":"7","author":"J Lansom","year":"2016","unstructured":"Lansom J, Alzahrani N, Liauw W, Morris DL. Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for pseudomyxoma peritonei and appendix tumours. Indian J Surg Oncol. 2016;7:166\u201376.","journal-title":"Indian J Surg Oncol"},{"key":"1723_CR48","first-page":"639","volume":"23","author":"M Vaira","year":"2009","unstructured":"Vaira M, Cioppa T, Marco GD, Bing C, D\u2019Amico S, D\u2019Alessandro M, et al. Management of pseudomyxoma peritonei by cytoreduction+HIPEC (hyperthermic intraperitoneal chemotherapy): results analysis of a twelve-year experience. Vivo. 2009;23:639\u201344.","journal-title":"Vivo"},{"key":"1723_CR49","doi-asserted-by":"publisher","first-page":"434","DOI":"10.1016\/j.jamcollsurg.2017.12.027","volume":"226","author":"EA Levine","year":"2018","unstructured":"Levine EA, Votanopoulos KI, Shen P, Russell G, Fenstermaker J, Mansfield P, et al. A multicenter randomized trial to evaluate hematologic toxicities after hyperthermic intraperitoneal chemotherapy with oxaliplatin or mitomycin in patients with appendiceal tumors. J Am Coll Surg. 2018;226:434\u201343.","journal-title":"J Am Coll Surg"},{"key":"1723_CR50","doi-asserted-by":"publisher","first-page":"439","DOI":"10.1007\/s10147-012-0397-5","volume":"18","author":"TC Chua","year":"2013","unstructured":"Chua TC, Liauw W, Zhao J, Morris DL. Comparative analysis of perioperative intraperitoneal chemotherapy regimen in appendiceal and colorectal peritoneal carcinomatosis. Int J Clin Oncol. 2013;18:439\u201346.","journal-title":"Int J Clin Oncol"},{"key":"1723_CR51","doi-asserted-by":"publisher","first-page":"176","DOI":"10.1245\/s10434-016-5529-0","volume":"24","author":"Y Huang","year":"2017","unstructured":"Huang Y, Alzahrani NA, Liauw W, Traiki TB, Morris DL. Early postoperative intraperitoneal chemotherapy for low-grade appendiceal mucinous neoplasms with pseudomyxoma peritonei: is it beneficial? Ann Surg Oncol. 2017;24:176\u201383.","journal-title":"Ann Surg Oncol"},{"key":"1723_CR52","doi-asserted-by":"publisher","first-page":"2292","DOI":"10.1016\/j.ejso.2017.09.002","volume":"43","author":"Y Huang","year":"2017","unstructured":"Huang Y, Alzahrani NA, Liauw W, Soudy H, Alzahrani AM, Morris DL. Early postoperative intraperitoneal chemotherapy is associated with survival benefit for appendiceal adenocarcinoma with peritoneal dissemination. Eur J Surg Oncol. 2017;43:2292\u20138.","journal-title":"Eur J Surg Oncol"},{"key":"1723_CR53","doi-asserted-by":"publisher","first-page":"424","DOI":"10.1016\/j.amjsurg.2015.03.008","volume":"210","author":"JY Lam","year":"2015","unstructured":"Lam JY, McConnell YJ, Rivard JD, Temple WJ, Mack LA. Hyperthermic intraperitoneal chemotherapy + early postoperative intraperitoneal chemotherapy versus hyperthermic intraperitoneal chemotherapy alone: assessment of survival outcomes for colorectal and high-grade appendiceal peritoneal carcinomatosis. Am J Surg. 2015;210:424\u201330.","journal-title":"Am J Surg"},{"key":"1723_CR54","doi-asserted-by":"publisher","first-page":"281","DOI":"10.3109\/02656736.2015.1135485","volume":"32","author":"GH Tan","year":"2016","unstructured":"Tan GH, Ong WS, Chia CS, Tham CK, Soo KC, Teo MC. Does early post-operative intraperitoneal chemotherapy (EPIC) for patients treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC) make a difference? Int J Hyperthermia. 2016;32:281\u20138.","journal-title":"Int J Hyperthermia"},{"key":"1723_CR55","unstructured":"ICARuS Post-operative Intraperitoneal Chemotherapy (EPIC) and Hyperthermic Intraperitoneal Chemotherapy (HIPEC) After Optimal Cytoreductive Surgery (CRS) for Neoplasms of the Appendix, Colon or Rectum With Isolated Peritoneal Metastasis (ICARuS). https:\/\/www.clinicaltrials.gov\/ct2\/show\/NCT01815359."},{"key":"1723_CR56","doi-asserted-by":"publisher","first-page":"243","DOI":"10.1097\/SLA.0b013e31818eec64","volume":"249","author":"D Baratti","year":"2009","unstructured":"Baratti D, Kusamura S, Nonaka D, Cabras AD, Laterza B, Deraco M. Pseudomyxoma peritonei: biological features are the dominant prognostic determinants after complete cytoreduction and hyperthermic intraperitoneal chemotherapy. Ann Surg. 2009;249:243\u20139.","journal-title":"Ann Surg"},{"key":"1723_CR57","doi-asserted-by":"publisher","first-page":"740","DOI":"10.1002\/jso.23547","volume":"109","author":"AU Blackham","year":"2014","unstructured":"Blackham AU, Swett K, Eng C, Sirintrapun J, Bergman S, Geisinger KR, et al. Perioperative systemic chemotherapy for appendiceal mucinous carcinoma peritonei treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. J Surg Oncol. 2014;109:740\u20135.","journal-title":"J Surg Oncol"},{"key":"1723_CR58","first-page":"163284","volume":"2012","author":"L Bijelic","year":"2012","unstructured":"Bijelic L, Kumar AS, Stuart OA, Sugarbaker PH. Systemic chemotherapy prior to cytoreductive surgery and HIPEC for carcinomatosis from appendix cancer: impact on perioperative outcomes and short-term survival. Gastroenterol Res Pract. 2012;2012:163284.","journal-title":"Gastroenterol Res Pract"},{"key":"1723_CR59","doi-asserted-by":"publisher","first-page":"652","DOI":"10.1093\/annonc\/mdr279","volume":"23","author":"CH Lieu","year":"2012","unstructured":"Lieu CH, Lambert LA, Wolff RA, Eng C, Zhang N, Wen S, et al. Systemic chemotherapy and surgical cytoreduction for poorly differentiated and signet ring cell adenocarcinomas of the appendix. Ann Oncol. 2012;23:652\u20138.","journal-title":"Ann Oncol"},{"key":"1723_CR60","doi-asserted-by":"publisher","first-page":"617","DOI":"10.1016\/j.surg.2012.07.027","volume":"152","author":"HL Van Sweringen","year":"2012","unstructured":"Van Sweringen HL, Hanseman DJ, Ahmad SA, Edwards MJ, Sussman JJ. Predictors of survival in patients with high-grade peritoneal metastases undergoing cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. Surgery. 2012;152:617\u201324 (discussion 24-5).","journal-title":"Surgery."},{"key":"1723_CR61","doi-asserted-by":"publisher","first-page":"1270","DOI":"10.1634\/theoncologist.2013-0186","volume":"18","author":"KP Raghav","year":"2013","unstructured":"Raghav KP, Shetty AV, Kazmi SM, Zhang N, Morris J, Taggart M, et al. Impact of molecular alterations and targeted therapy in appendiceal adenocarcinomas. Oncologist. 2013;18:1270\u20137.","journal-title":"Oncologist"},{"key":"1723_CR62","doi-asserted-by":"publisher","first-page":"1068","DOI":"10.1245\/s10434-012-2789-1","volume":"20","author":"KM Turner","year":"2013","unstructured":"Turner KM, Hanna NN, Zhu Y, Jain A, Kesmodel SB, Switzer RA, et al. Assessment of neoadjuvant chemotherapy on operative parameters and outcome in patients with peritoneal dissemination from high-grade appendiceal cancer. Ann Surg Oncol. 2013;20:1068\u201373.","journal-title":"Ann Surg Oncol"},{"key":"1723_CR63","doi-asserted-by":"publisher","first-page":"707","DOI":"10.1016\/j.ejso.2015.01.005","volume":"41","author":"V Milovanov","year":"2015","unstructured":"Milovanov V, Sardi A, Ledakis P, Aydin N, Nieroda C, Sittig M, et al. Systemic chemotherapy (SC) before cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS\/HIPEC) in patients with peritoneal mucinous carcinomatosis of appendiceal origin (PMCA). Eur J Surg Oncol. 2015;41:707\u201312.","journal-title":"Eur J Surg Oncol"},{"key":"1723_CR64","first-page":"783","volume":"22","author":"J Spiliotis","year":"2017","unstructured":"Spiliotis J, Kopanakis N, Efstathiou E, Vassiliadou D, Argiriou O, Rogdakis A, et al. Perioperative systemic chemotherapy for peritoneal mucinous appendiceal carcinomas treated with cytoreductive surgery & HIPEC. J BUON. 2017;22:783\u20139.","journal-title":"J BUON"},{"key":"1723_CR65","doi-asserted-by":"publisher","first-page":"213","DOI":"10.1002\/cncr.29744","volume":"122","author":"EA Asare","year":"2016","unstructured":"Asare EA, Compton CC, Hanna NN, Kosinski LA, Washington MK, Kakar S, et al. The impact of stage, grade, and mucinous histology on the efficacy of systemic chemotherapy in adenocarcinomas of the appendix: analysis of the National Cancer Data Base. Cancer. 2016;122:213\u201321.","journal-title":"Cancer"},{"key":"1723_CR66","doi-asserted-by":"publisher","first-page":"588","DOI":"10.1097\/SLA.0b013e31819ec7e3","volume":"249","author":"DA Schomas","year":"2009","unstructured":"Schomas DA, Miller RC, Donohue JH, Gill S, Thurmes PJ, Haddock MG, et al. Intraperitoneal treatment for peritoneal mucinous carcinomatosis of appendiceal origin after operative management: long-term follow-up of the Mayo Clinic experience. Ann Surg. 2009;249:588\u201395.","journal-title":"Ann Surg"},{"key":"1723_CR67","first-page":"3","volume":"7","author":"KA Cummins","year":"2016","unstructured":"Cummins KA, Russell GB, Votanopoulos KI, Shen P, Stewart JH, Levine EA. Peritoneal dissemination from high-grade appendiceal cancer treated with cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC). J Gastrointest Oncol. 2016;7:3\u20139.","journal-title":"J Gastrointest Oncol"}],"container-title":["Orphanet Journal of Rare Diseases"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/s13023-021-01723-6.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1186\/s13023-021-01723-6\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/s13023-021-01723-6.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,2,13]],"date-time":"2021-02-13T20:46:48Z","timestamp":1613249208000},"score":1,"resource":{"primary":{"URL":"https:\/\/ojrd.biomedcentral.com\/articles\/10.1186\/s13023-021-01723-6"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,2,13]]},"references-count":67,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2021,12]]}},"alternative-id":["1723"],"URL":"https:\/\/doi.org\/10.1186\/s13023-021-01723-6","relation":{},"ISSN":["1750-1172"],"issn-type":[{"value":"1750-1172","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,2,13]]},"assertion":[{"value":"21 September 2020","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"2 February 2021","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"13 February 2021","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"Not applicable.","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Not applicable.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare that they have no competing interests.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"85"}}