{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,18]],"date-time":"2026-05-18T05:23:14Z","timestamp":1779081794691,"version":"3.51.4"},"reference-count":30,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2018,7,26]],"date-time":"2018-07-26T00:00:00Z","timestamp":1532563200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Colorectal Disease"],"published-print":{"date-parts":[[2018,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Aim<\/jats:title><jats:p>Pseudomyxoma peritonei (<jats:styled-content style=\"fixed-case\">PMP<\/jats:styled-content>) is a rare neoplasm of the appendix, which if untreated disseminates throughout the abdominal cavity and generates considerable morbidity. Since 2002 in the <jats:styled-content style=\"fixed-case\">UK<\/jats:styled-content>, patients with <jats:styled-content style=\"fixed-case\">PMP<\/jats:styled-content> have been managed via two nationally commissioned centres. We evaluated referrals and treatment pathways over time at the Manchester centre.<\/jats:p><\/jats:sec><jats:sec><jats:title>Method<\/jats:title><jats:p>Data from all patients referred with suspected <jats:styled-content style=\"fixed-case\">PMP<\/jats:styled-content> were prospectively collected (2002\u20132015). Definitive treatment was cytoreductive surgery (<jats:styled-content style=\"fixed-case\">CRS<\/jats:styled-content>) and hyperthermic intraperitoneal chemotherapy. Disease burden was quantified using the Peritoneal Cancer Index (<jats:styled-content style=\"fixed-case\">PCI<\/jats:styled-content>) (score 0\u201339) and complete cytoreduction (<jats:styled-content style=\"fixed-case\">CC<\/jats:styled-content>) defined by scores of 0\/1. Novel treatment algorithms were developed for patients with low grade appendiceal mucinous neoplasm (<jats:styled-content style=\"fixed-case\">LAMN<\/jats:styled-content>) localized to the peri\u2010appendiceal tissue.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In all, 817 patients with confirmed <jats:styled-content style=\"fixed-case\">PMP<\/jats:styled-content> were referred increasing from 11 in 2002 to 103 in 2015. Disease burden was high with a mean <jats:styled-content style=\"fixed-case\">PCI<\/jats:styled-content> of 31 in the first quartile (Q1), levelling off to 15, 15, 17 thereafter (<jats:italic>P\u00a0<\/jats:italic>=<jats:italic>\u00a0<\/jats:italic>0.002). The proportion of <jats:styled-content style=\"fixed-case\">CC<\/jats:styled-content>0\/1 increased from 67% in Q1 to 77% Q2 and 74% Q3\/4. Where complete cytoreduction was achieved, 5\u2010 and 10\u2010year overall survival was 77% and 66%. The proportion of patients referred with localized <jats:styled-content style=\"fixed-case\">LAMN<\/jats:styled-content> increased over time reaching 25% each year since 2010 (<jats:italic>P<\/jats:italic><jats:sub>trend<\/jats:sub>\u00a0&lt;\u00a00.0001). Two\u2010thirds of localized <jats:styled-content style=\"fixed-case\">LAMN<\/jats:styled-content> now undergo laparoscopically assisted risk\u2010reducing <jats:styled-content style=\"fixed-case\">CRS<\/jats:styled-content>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The establishment of a national treatment centre was associated with an initial presentation of patients with advanced disease. The programme has demonstrated a clear trend over time towards earlier referral and adoption of minimally invasive techniques for localized disease.<\/jats:p><\/jats:sec>","DOI":"10.1111\/codi.14310","type":"journal-article","created":{"date-parts":[[2018,6,19]],"date-time":"2018-06-19T19:13:58Z","timestamp":1529435638000},"page":"888-896","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Referral and treatment pathways for pseudomyxoma peritonei of appendiceal origin within a national treatment programme"],"prefix":"10.1111","volume":"20","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4239-6993","authenticated-orcid":false,"given":"R.","family":"Fish","sequence":"first","affiliation":[{"name":"Colorectal and Peritoneal Oncology Centre Christie NHS Foundation Trust Manchester UK"},{"name":"Division of Cancer Sciences School of Medical Sciences Faculty of Biology, Medicine and Health Manchester Academic Health Science Centre University of Manchester Manchester UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A. G.","family":"Renehan","sequence":"additional","affiliation":[{"name":"Colorectal and Peritoneal Oncology Centre Christie NHS Foundation Trust Manchester UK"},{"name":"Division of Cancer Sciences School of Medical Sciences Faculty of Biology, Medicine and Health Manchester Academic Health Science Centre University of Manchester Manchester UK"},{"name":"Manchester Cancer Research Centre NIHR Manchester Biomedical Research Centre University of Manchester Manchester UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G.","family":"Punnett","sequence":"additional","affiliation":[{"name":"Colorectal and Peritoneal Oncology Centre Christie NHS Foundation Trust Manchester UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"O.","family":"Aziz","sequence":"additional","affiliation":[{"name":"Colorectal and Peritoneal Oncology Centre Christie NHS Foundation Trust Manchester UK"},{"name":"Division of Cancer Sciences School of Medical Sciences Faculty of Biology, Medicine and Health Manchester Academic Health Science Centre University of Manchester Manchester UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"P.","family":"Fulford","sequence":"additional","affiliation":[{"name":"Colorectal and Peritoneal Oncology Centre Christie NHS Foundation Trust Manchester UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C.","family":"Selvasekar","sequence":"additional","affiliation":[{"name":"Colorectal and Peritoneal Oncology Centre Christie NHS Foundation Trust Manchester UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Wilson","sequence":"additional","affiliation":[{"name":"Colorectal and Peritoneal Oncology Centre Christie NHS Foundation Trust Manchester UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R.","family":"Halstead","sequence":"additional","affiliation":[{"name":"Colorectal and Peritoneal Oncology Centre Christie NHS Foundation Trust Manchester UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S. T.","family":"O'Dwyer","sequence":"additional","affiliation":[{"name":"Colorectal and Peritoneal Oncology Centre Christie NHS Foundation Trust Manchester UK"},{"name":"Division of Cancer Sciences School of Medical Sciences Faculty of Biology, Medicine and Health Manchester Academic Health Science Centre University of Manchester Manchester UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2018,7,26]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-3-540-47648-1_4843"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00384-013-1714-9"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1470-2045(05)70539-8"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2011.39.7166"},{"key":"e_1_2_8_6_1","unstructured":"NHS Commissioning Board.Pseudomyxoma peritonei service (Adult) Service Specification A08\/S(HSS)\/b 2013.https:\/\/www.england.nhs.uk\/wp-content\/uploads\/2013\/06\/a08-pseud-peri-ad.pdf. 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