{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,13]],"date-time":"2026-09-13T05:00:50Z","timestamp":1789275650617,"version":"build-2803163510"},"reference-count":43,"publisher":"American Medical Association (AMA)","issue":"5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JAMA Surg"],"published-print":{"date-parts":[[2023,5,1]]},"abstract":"Importance<\/jats:title>Pseudomyxoma peritoni, a rare condition characterized by mucinous ascites and peritoneal deposits, mainly originates from a ruptured mucinous appendix tumor and is considered an indolent disease but can progress and become fatal. Optimal treatment to improve cure and survival rates involves complete cytoreductive surgery (CCRS) and hyperthermic intraperitoneal chemotherapy (HIPEC). Accurate predictive models are useful in supporting and informing treatment strategies and stratifying patient follow-up.<\/jats:p><\/jats:sec>Objective<\/jats:title>To evaluate the prognostic significance of clinically important variables and generate validated nomograms to predict overall (OS) and disease-free survival (DFS) following CCRS and hyperthermic intraperitoneal HIPEC for pseudomyxoma peritonei (PMP) of appendiceal origin.<\/jats:p><\/jats:sec>Design, Setting, and Participants<\/jats:title>This retrospective study used prospectively collected data on patients who had cytoreductive surgery (CRS) and HIPEC in a single institution between 1994 and 2018. The cohort was randomly allocated into development (70%) and validation (30%) sets. Univariate and multivariate analyses were performed with Cox proportional hazards regression.<\/jats:p><\/jats:sec>Main Outcomes and Measures<\/jats:title>A prediction model was developed with significant prognostic factors identified by multivariate analysis. The model\u2019s prognostic performance was evaluated with the concordance index (C index). The nomogram was calibrated by comparing the predicted and observed probabilities.<\/jats:p><\/jats:sec>Results<\/jats:title>Of 2637 CRS and HIPEC operations, 1102 patients (female, 64.4%; median age [IQR], 57.0 [48.0-66.0] years) (41.8%) had CCRS for PMP of appendiceal origin. Elevated tumor markers, peritoneal carcinomatosis index, gastrectomy, and tumor grade were independent predictive factors for DFS. Gender, age, elevated tumor makers, peritoneal carcinomatosis index, and tumor grade influenced OS. The nomograms were generated with respective prognostic factors. The nomograms showed good performance in predicting survival. Median OS of the cohort was 16.5 years (95% CI, 13.7-19.2) with a 5-year probability of survival of 80.2%. The median DFS was 10.3 years (95% CI, 7.2- 13.3) and the 5-year probability of recurrence-free survival was 60.5%.<\/jats:p><\/jats:sec>Conclusions and Relevance<\/jats:title>Clinically important independent predictors for survival and recurrence were selected to develop the nomograms for OS and DFS. These 2 nomograms are user friendly and useful tools for patient management with clinical trial design applications.<\/jats:p><\/jats:sec>","DOI":"10.1001\/jamasurg.2023.0112","type":"journal-article","created":{"date-parts":[[2023,3,15]],"date-time":"2023-03-15T15:07:38Z","timestamp":1678892858000},"page":"522","source":"Crossref","is-referenced-by-count":28,"title":["Development and Validation of Nomograms to Predict Survival in Patients Undergoing Complete Cytoreduction and Hyperthermic Intraperitoneal Chemotherapy for Pseudomyxoma Peritonei of Appendiceal Origin"],"prefix":"10.1001","volume":"158","author":[{"given":"Kandiah","family":"Chandrakumaran","sequence":"first","affiliation":[{"name":"Peritoneal Malignancy Institute, Basingstoke and North Hampshire Hospital, Basingstoke, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Norman John","family":"Carr","sequence":"additional","affiliation":[{"name":"Peritoneal Malignancy Institute, Basingstoke and North Hampshire Hospital, Basingstoke, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Faheez","family":"Mohamed","sequence":"additional","affiliation":[{"name":"Peritoneal Malignancy Institute, Basingstoke and North Hampshire Hospital, Basingstoke, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas Desmond","family":"Cecil","sequence":"additional","affiliation":[{"name":"Peritoneal Malignancy Institute, Basingstoke and North Hampshire Hospital, Basingstoke, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brendan John","family":"Moran","sequence":"additional","affiliation":[{"name":"Peritoneal Malignancy Institute, Basingstoke and North Hampshire Hospital, Basingstoke, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"10","reference":[{"key":"soi230003r1","doi-asserted-by":"publisher","first-page":"105","DOI":"10.1007\/978-1-4613-1245-1","article-title":"Pseudomyxoma peritonei.","volume":"81","author":"Sugarbaker","year":"1996","journal-title":"Cancer Treat Res"},{"issue":"3","key":"soi230003r2","doi-asserted-by":"publisher","first-page":"585","DOI":"10.1016\/S1055-3207(03)00026-7","article-title":"The etiology, clinical presentation, and management of pseudomyxoma peritonei.","volume":"12","author":"Moran","year":"2003","journal-title":"Surg Oncol Clin N Am"},{"issue":"1","key":"soi230003r3","doi-asserted-by":"publisher","first-page":"252","DOI":"10.1245\/s10434-020-08655-8","article-title":"Estimating the prevalence of pseudomyxoma peritonei in Europe using a novel statistical method.","volume":"28","author":"Patrick-Brown","year":"2020","journal-title":"Ann Surg Oncol"},{"issue":"1","key":"soi230003r4","doi-asserted-by":"publisher","first-page":"29","DOI":"10.1097\/00000658-199501000-00004","article-title":"Peritonectomy procedures.","volume":"221","author":"Sugarbaker","year":"1995","journal-title":"Ann Surg"},{"issue":"7","key":"soi230003r6","doi-asserted-by":"publisher","first-page":"1035","DOI":"10.1016\/j.ejso.2016.03.017","article-title":"Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy in 1000 patients with perforated appendiceal epithelial tumours.","volume":"42","author":"Ansari","year":"2016","journal-title":"Eur J Surg Oncol"},{"issue":"3","key":"soi230003r7","doi-asserted-by":"publisher","first-page":"376","DOI":"10.1002\/bjs5.2019.3.issue-3","article-title":"Factors influencing long-term survival after cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for pseudomyxoma peritonei originating from appendiceal neoplasms.","volume":"3","author":"van Eden","year":"2019","journal-title":"BJS Open"},{"issue":"2","key":"soi230003r8","doi-asserted-by":"publisher","first-page":"484","DOI":"10.1245\/s10434-006-9182-x","article-title":"A systematic review on the efficacy of cytoreductive surgery and perioperative intraperitoneal chemotherapy for pseudomyxoma peritonei.","volume":"14","author":"Yan","year":"2007","journal-title":"Ann Surg Oncol"},{"issue":"5","key":"soi230003r9","doi-asserted-by":"publisher","first-page":"456","DOI":"10.1016\/j.ejso.2010.01.006","article-title":"Pseudomyxoma peritonei: a French multicentric study of 301 patients treated with cytoreductive surgery and intraperitoneal chemotherapy.","volume":"36","author":"Elias","year":"2010","journal-title":"Eur J Surg Oncol"},{"issue":"2","key":"soi230003r10","doi-asserted-by":"publisher","first-page":"300","DOI":"10.1097\/01.sla.0000152015.76731.1f","article-title":"Long-term survival following treatment of pseudomyxoma peritonei: an analysis of surgical therapy.","volume":"241","author":"Miner","year":"2005","journal-title":"Ann Surg"},{"issue":"2","key":"soi230003r11","first-page":"173","article-title":"Carbohydrate antigen 19-9 (CA 19-9) is an independent prognostic indicator in pseudomyxoma peritonei post cytoreductive surgery and perioperative intraperitoneal chemotherapy.","volume":"4","author":"Koh","year":"2013","journal-title":"J Gastrointest Oncol"},{"issue":"5","key":"soi230003r12","doi-asserted-by":"publisher","first-page":"515","DOI":"10.1016\/j.ejso.2013.12.021","article-title":"Pre-operative tumour marker status predicts recurrence and survival after complete cytoreduction and hyperthermic intraperitoneal chemotherapy for appendiceal pseudomyxoma peritonei: analysis of 519 patients.","volume":"40","author":"Taflampas","year":"2014","journal-title":"Eur J Surg Oncol"},{"issue":"3","key":"soi230003r13","doi-asserted-by":"publisher","first-page":"225","DOI":"10.1097\/PPO.0b013e3181a9c781","article-title":"Epithelial appendiceal neoplasms.","volume":"15","author":"Sugarbaker","year":"2009","journal-title":"Cancer J"},{"issue":"13","key":"soi230003r14","doi-asserted-by":"publisher","first-page":"4316","DOI":"10.1245\/s10434-016-5389-7","article-title":"Advanced pseudomyxoma peritonei requiring gastrectomy to achieve complete cytoreduction results in good long-term oncologic outcomes.","volume":"23","author":"Di Fabio","year":"2016","journal-title":"Ann Surg Oncol"},{"issue":"9","key":"soi230003r15","doi-asserted-by":"publisher","first-page":"1371","DOI":"10.1016\/j.ejso.2018.06.023","article-title":"Single centre guidelines for radiological follow-up based on 775 patients treated by cytoreductive surgery and HIPEC for appendiceal pseudomyxoma peritonei.","volume":"44","author":"Govaerts","year":"2018","journal-title":"Eur J Surg Oncol"},{"issue":"4","key":"soi230003r16","doi-asserted-by":"publisher","first-page":"878","DOI":"10.1245\/s10434-017-6305-5","article-title":"Clinical surveillance after macroscopically complete surgery for low-grade appendiceal mucinous neoplasms (LAMN) with or without limited peritoneal spread: long-term results in a prospective series.","volume":"25","author":"Guaglio","year":"2018","journal-title":"Ann Surg Oncol"},{"key":"soi230003r17","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejso.2020.02.012","article-title":"Appendiceal tumours and pseudomyxoma peritonei: literature review with PSOGI\/EURACAN clinical practice guidelines for diagnosis and treatment.","volume":"47","author":"Govaerts","year":"2020","journal-title":"Eur J Surg Oncol"},{"issue":"8","key":"soi230003r19","doi-asserted-by":"publisher","first-page":"1364","DOI":"10.1200\/JCO.2007.12.9791","article-title":"How to build and interpret a nomogram for cancer prognosis.","volume":"26","author":"Iasonos","year":"2008","journal-title":"J Clin Oncol"},{"issue":"2","key":"soi230003r20","doi-asserted-by":"publisher","first-page":"282","DOI":"10.1097\/SLA.0b013e31815ed67b","article-title":"A nomogram for predicting disease-specific survival after hepatic resection for metastatic colorectal cancer.","volume":"247","author":"Kattan","year":"2008","journal-title":"Ann Surg"},{"issue":"16","key":"soi230003r21","doi-asserted-by":"publisher","first-page":"2044","DOI":"10.1016\/S0959-8049(00)00286-0","article-title":"A clinical nomogram for predicting long-term survival in advanced colorectal cancer.","volume":"36","author":"Massacesi","year":"2000","journal-title":"Eur J Cancer"},{"issue":"33","key":"soi230003r22","doi-asserted-by":"publisher","first-page":"8331","DOI":"10.1200\/JCO.2005.01.2898","article-title":"Nomograms to predict pathologic complete response and metastasis-free survival after preoperative chemotherapy for breast cancer.","volume":"23","author":"Rouzier","year":"2005","journal-title":"J Clin Oncol"},{"issue":"2","key":"soi230003r23","doi-asserted-by":"publisher","first-page":"215","DOI":"10.1016\/j.radonc.2014.11.002","article-title":"Nomogram predicting response after chemoradiotherapy in rectal cancer using sequential PETCT imaging: a multicentric prospective study with external validation.","volume":"113","author":"van Stiphout","year":"2014","journal-title":"Radiother Oncol"},{"key":"soi230003r24","doi-asserted-by":"publisher","first-page":"359","DOI":"10.1007\/978-1-4613-1247-5","article-title":"Clinical research methodologies in diagnosis and staging of patients with peritoneal carcinomatosis.","volume":"82","author":"Jacquet","year":"1996","journal-title":"Cancer Treat Res"},{"issue":"2","key":"soi230003r25","doi-asserted-by":"publisher","first-page":"187","DOI":"10.1097\/SLA.0b013e3181b13ca2","article-title":"The Clavien-Dindo classification of surgical complications: five-year experience.","volume":"250","author":"Clavien","year":"2009","journal-title":"Ann Surg"},{"issue":"1","key":"soi230003r26","doi-asserted-by":"publisher","first-page":"14","DOI":"10.1097\/PAS.0000000000000535","article-title":"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.","volume":"40","author":"Carr","year":"2016","journal-title":"Am J Surg Pathol"},{"issue":"6","key":"soi230003r27","doi-asserted-by":"publisher","first-page":"847","DOI":"10.1111\/his.2017.71.issue-6","article-title":"The histopathological classification, diagnosis and differential diagnosis of mucinous appendiceal neoplasms, appendiceal adenocarcinomas and pseudomyxoma peritonei.","volume":"71","author":"Carr","year":"2017","journal-title":"Histopathology"},{"issue":"4","key":"soi230003r28","doi-asserted-by":"crossref","first-page":"795","DOI":"10.1177\/1536867X1301300407","article-title":"Littles test of missing completely at random.","volume":"13","author":"Li","year":"2013","journal-title":"Stata J"},{"issue":"404","key":"soi230003r29","doi-asserted-by":"crossref","first-page":"1198","DOI":"10.1080\/01621459.1988.10478722","article-title":"A test of missing completely at random for multivariate data with missing values.","volume":"83","author":"Little","year":"1998","journal-title":"Journal of the American Statistical Association"},{"issue":"3","key":"soi230003r30","doi-asserted-by":"crossref","DOI":"10.18637\/jss.v045.i03","article-title":"mice: Multivariate imputation by chained equations in R.","volume":"45","author":"Buuren","year":"2011","journal-title":"J Stat Softw"},{"issue":"18","key":"soi230003r32","doi-asserted-by":"crossref","first-page":"2543","DOI":"10.1001\/jama.1982.03320430047030","article-title":"Evaluating the yield of medical tests.","volume":"247","author":"Harrell","year":"1982","journal-title":"JAMA"},{"issue":"4","key":"soi230003r33","doi-asserted-by":"crossref","first-page":"361","DOI":"10.1002\/(SICI)1097-0258(19960229)15:4<361::AID-SIM168>3.0.CO;2-4","article-title":"Multivariable prognostic models: issues in developing models, evaluating assumptions and adequacy, and measuring and reducing errors.","volume":"15","author":"Harrell","year":"1996","journal-title":"Stat Med"},{"issue":"13","key":"soi230003r34","doi-asserted-by":"crossref","first-page":"1758","DOI":"10.1002\/bjs.9674","article-title":"Multicentre study of the learning curve and surgical performance of cytoreductive surgery with intraperitoneal chemotherapy for pseudomyxoma peritonei.","volume":"101","author":"Kusamura","year":"2014","journal-title":"Br J Surg"},{"issue":"2","key":"soi230003r35","doi-asserted-by":"crossref","first-page":"104","DOI":"10.3322\/caac.21220","article-title":"Colorectal cancer statistics, 2014.","volume":"64","author":"Siegel","year":"2014","journal-title":"CA Cancer J Clin"},{"issue":"10","key":"soi230003r36","doi-asserted-by":"crossref","first-page":"1942","DOI":"10.1002\/ijc.30827","article-title":"Gender differences in colorectal cancer survival: a meta-analysis.","volume":"141","author":"Yang","year":"2017","journal-title":"Int J Cancer"},{"issue":"8","key":"soi230003r37","doi-asserted-by":"crossref","first-page":"704","DOI":"10.1111\/codi.14057","article-title":"Impact of ovarian metastases on survival in patients treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for peritoneal malignancy originating from appendiceal and colorectal cancer.","volume":"20","author":"Bignell","year":"2018","journal-title":"Colorectal Dis"},{"issue":"10","key":"soi230003r38","doi-asserted-by":"crossref","first-page":"1358","DOI":"10.1038\/bjc.2017.96","article-title":"Serum inflammatory markers and colorectal cancer risk and survival.","volume":"116","author":"Ghuman","year":"2017","journal-title":"Br J Cancer"},{"issue":"4","key":"soi230003r39","doi-asserted-by":"crossref","first-page":"667","DOI":"10.1177\/0194599817745284","article-title":"Correlation of inflammatory markers, survival, and COX2 expression in oral cancer and implications for prognosis.","volume":"158","author":"Sano","year":"2018","journal-title":"Otolaryngol Head Neck Surg"},{"issue":"2","key":"soi230003r40","doi-asserted-by":"crossref","first-page":"388","DOI":"10.1016\/j.ejso.2016.10.009","article-title":"The modified Glasgow prognosis score predicts for overall and disease-free survival following cytoreductive surgery and HIPEC in patients with pseudomyxoma peritonei of appendiceal origin.","volume":"43","author":"Tan","year":"2017","journal-title":"Eur J Surg Oncol"},{"issue":"5","key":"soi230003r41","doi-asserted-by":"crossref","first-page":"559","DOI":"10.1080\/02656736.2017.1384073","article-title":"The pre-operative neutrophil-lymphocyte ratio predicts overall and disease-free survival following cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) in patients with pseudomxyoma peritonei of appendiceal origin.","volume":"34","author":"Rangarajan","year":"2018","journal-title":"Int J Hyperthermia"},{"key":"soi230003r42","doi-asserted-by":"crossref","first-page":"584","DOI":"10.3389\/fonc.2019.00584","article-title":"A Simple-to-use nomogram for predicting the survival of early hepatocellular carcinoma patients.","volume":"9","author":"Chen","year":"2019","journal-title":"Front Oncol"},{"issue":"1","key":"soi230003r43","doi-asserted-by":"crossref","first-page":"220","DOI":"10.1002\/ijc.30071","article-title":"Nomograms for predicting prognostic value of inflammatory biomarkers in colorectal cancer patients after radical resection.","volume":"139","author":"Li","year":"2016","journal-title":"Int J Cancer"},{"key":"soi230003r5","volume-title":"Intraperitoneal Chemotherapy and Cytoreductive Surgery: Manual for Physicians and Nurses","author":"Sugarbaker","year":"1998","edition":"3rd ed"},{"key":"soi230003r18","doi-asserted-by":"crossref","DOI":"10.1007\/978-0-387-77244-8","volume-title":"Clinical Prediction Models","author":"Steyerberg","year":"2009"},{"key":"soi230003r31","doi-asserted-by":"crossref","DOI":"10.1007\/978-1-4757-3462-1","volume-title":"Regression modelling strategies: with applications to linear models, logistic regression, and survival analysis. Springer series in statistics","author":"Harrell","year":"2001"}],"container-title":["JAMA Surgery"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/jamanetwork.com\/journals\/jamasurgery\/articlepdf\/2802653\/jamasurgery_chandrakumaran_2023_oi_230003_1683554080.3003.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,5,10]],"date-time":"2023-05-10T17:20:39Z","timestamp":1683739239000},"score":1,"resource":{"primary":{"URL":"https:\/\/jamanetwork.com\/journals\/jamasurgery\/fullarticle\/2802653"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,5,1]]},"references-count":43,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2023,5,1]]}},"URL":"https:\/\/doi.org\/10.1001\/jamasurg.2023.0112","relation":{},"ISSN":["2168-6254"],"issn-type":[{"value":"2168-6254","type":"print"}],"subject":[],"published":{"date-parts":[[2023,5,1]]}}}