{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T20:22:03Z","timestamp":1790713323177,"version":"4.1.0"},"reference-count":39,"publisher":"MDPI AG","issue":"11","license":[{"start":{"date-parts":[[2024,10,29]],"date-time":"2024-10-29T00:00:00Z","timestamp":1730160000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Life"],"abstract":"<jats:p>Objective: Pseudomyxoma peritonei (PP) is a rare condition, and differentiating between primary and secondary ovarian causes is crucial for determining the appropriate oncological therapy. Given the resistance of ovarian mucinous carcinoma to standard platinum-based chemotherapy, the objective of this review is to present the current therapeutic approaches and summarize the emerging trends in the treatment of this disease. Methods: The authors conducted an exhaustive evaluation of studies published over a 14-year period (June 2010\u2013May 2024) concerning pseudomyxoma peritonei, mucinous ovarian carcinoma, ovarian causes of PP, and ovarian cancer using the following databases: PubMed, Scopus, and Science Direct. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were followed. The results were organized into seven subchapters and analyzed. Results: The analyzed studies present surgery followed by HIPEC as the current therapy with the best long-term survival results. However, the oncological treatment is unsatisfactory, and the choice of therapy depending on the primary origin of the tumor becomes particularly important. For the differential diagnosis between pseudomyxoma due to a gastrointestinal cause and that of ovarian origin, genetic analyses are recommended; these include the characteristics of the mucin present in the lesion, as the therapeutic response can have contradictory results depending on the primary origin of the tumor. Conclusions: Surgery followed by HIPEC remains the standard for resectable cases. However, oncological treatment has controversial results in the case of mucinous ovarian carcinoma compared to other types of ovarian cancer and to metastatic ovarian tumors associated with pseudomyxoma of the peritoneum. Based on the articles included in this review, it was found that the current trend is the study of mucin as a resistance factor against chemotherapy based on platinum products and the targeting of oncological therapy according to the tumor\u2019s genetic characteristics.<\/jats:p>","DOI":"10.3390\/life14111390","type":"journal-article","created":{"date-parts":[[2024,10,29]],"date-time":"2024-10-29T10:45:35Z","timestamp":1730198735000},"page":"1390","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Status of and Challenges in Therapy of Mucinous Ovarian Cancer Associated with Pseudomyxoma Peritonei Syndrome: Review of Current Options and Future Treatment Trends"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2454-8110","authenticated-orcid":false,"given":"George","family":"Pariza","sequence":"first","affiliation":[{"name":"5th Department of General Surgery, Emergency Hospital Bucharest, 050098 Bucharest, Romania"},{"name":"Faculty of Medicine, \u201cCarol Davila\u201d University of Medicine and Pharmacy, 050474 Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carmen","family":"Mavrodin","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, \u201cCarol Davila\u201d University of Medicine and Pharmacy, 050474 Bucharest, Romania"},{"name":"3th Department of General Surgery, Emergency Hospital Bucharest, 050098 Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alina","family":"Potorac","sequence":"additional","affiliation":[{"name":"Department of Obstetrics and Gynecology, Emergency Hospital Bucharest, 050098 Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3438-1211","authenticated-orcid":false,"given":"Octavian","family":"Munteanu","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, \u201cCarol Davila\u201d University of Medicine and Pharmacy, 050474 Bucharest, Romania"},{"name":"Department of Obstetrics and Gynecology, Emergency Hospital Bucharest, 050098 Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Monica Mihaela","family":"Cirstoiu","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, \u201cCarol Davila\u201d University of Medicine and Pharmacy, 050474 Bucharest, Romania"},{"name":"Department of Obstetrics and Gynecology, Emergency Hospital Bucharest, 050098 Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,10,29]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"7569","DOI":"10.2147\/CMAR.S264474","article-title":"Clinicopathological Characteristics of Pseudomyxoma Peritonei Originated from Ovaries","volume":"12","author":"Yan","year":"2020","journal-title":"Cancer Manag. Res."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"511","DOI":"10.1080\/02656736.2017.1310938","article-title":"Pseudomyxoma peritonei: Natural history and treatment","volume":"33","author":"Mittal","year":"2017","journal-title":"Int. J. Hyperth."},{"key":"ref_3","unstructured":"Placek, A., and Pezhouh, M.K. (2024, September 13). Pseudomyxoma Peritonei\/Mucinous Carcinoma Peritonei. PathologyOutlines.com.website. Available online: https:\/\/www.pathologyoutlines.com\/topic\/appendixpseudomyxoma.html."},{"key":"ref_4","unstructured":"Yu, B., and Raj, M.S. (2024, September 13). Pseudomyxoma Peritonei, StatPearls [Internet], Available online: https:\/\/www.ncbi.nlm.nih.gov\/books\/NBK541116\/."},{"key":"ref_5","doi-asserted-by":"crossref","unstructured":"Ionescu, S., Marincas, M., Madge, O.L., Dicu-Andreescu, I.G., Chitoran, E., Rotaru, V., Cirimbei, C., Gherghe, M., Ene, A., and Rosca, R. (2024). Ovarian Causes of Pseudomyxoma Peritonei (PMP)-A Literature Review. Cancers, 16.","DOI":"10.3390\/cancers16081446"},{"key":"ref_6","doi-asserted-by":"crossref","unstructured":"Blaj, S., Dora, D., Lohinai, Z., Herold, Z., Szasz, A.M., Herzberg, J., Kodacsi, R., Baransi, S., Schlitt, H.J., and Hornung, M. (2023). Prognostic Factors in Pseudomyxoma Peritonei with Emphasis on the Predictive Role of Peritoneal Cancer Index and Tumor Markers. Cancers, 15.","DOI":"10.3390\/cancers15041326"},{"key":"ref_7","doi-asserted-by":"crossref","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."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1414","DOI":"10.1046\/j.1365-2168.2000.01553.x","article-title":"Clinical presentation of the Pseudomyxoma peritonei syndrome","volume":"87","author":"Esquivel","year":"2000","journal-title":"Br. J. Surg."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"1212","DOI":"10.1016\/j.ejso.2006.04.013","article-title":"MRI evaluation of bulky tumor masses in the mesentery and bladder involvement in peritoneal carcinomatosis","volume":"32","author":"Cotton","year":"2006","journal-title":"Eur. J. Surg. Oncol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1102\/1470-7330.2013.0003","article-title":"Mucinous neoplasms of the appendix: A current comprehensive clinicopathologic and imaging review","volume":"13","author":"Tirumani","year":"2013","journal-title":"Cancer Imaging"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"656","DOI":"10.2214\/AJR.07.2018","article-title":"Mucinous appendiceal neoplasms: Preoperative MR staging and classification compared with surgical and histopathologic findings","volume":"190","author":"Low","year":"2008","journal-title":"AJR Am. J. Roentgenol."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"558","DOI":"10.1016\/j.ejso.2016.01.005","article-title":"Preoperative CT and MRI prediction of non-resectability in patients treated for pseudomyxoma peritonei from mucinous appendiceal neoplasms","volume":"42","author":"Menassel","year":"2016","journal-title":"Eur. J. Surg. Oncol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"20210117","DOI":"10.1259\/bjr.20210117","article-title":"The role of CT, PET-CT, and MRI in ovarian cancer","volume":"94","author":"Engbersen","year":"2021","journal-title":"Br. J. Radiol."},{"key":"ref_14","first-page":"233","article-title":"Pseudomyxoma peritonei syndrome","volume":"30","author":"Sugarbaker","year":"1996","journal-title":"Adv. Surg."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"2177","DOI":"10.1245\/s10434-024-14892-y","article-title":"AJCC Cancer Staging System Version 9: Appendiceal Adenocarcinoma","volume":"31","author":"Hanna","year":"2024","journal-title":"Ann. Surg. Oncol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"e19221","DOI":"10.1016\/j.heliyon.2023.e19221","article-title":"Mucins and mucinous ovarian carcinoma: Development, differential diagnosis, and treatment","volume":"9","author":"Wang","year":"2023","journal-title":"Heliyon"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"87","DOI":"10.3802\/jgo.2015.26.2.87","article-title":"FIGO\u2019s staging classification for cancer of the ovary, fallopian tube, and peritoneum: Abridged republication","volume":"26","author":"Prat","year":"2015","journal-title":"J. Gynecol. Oncol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1016\/j.ygyno.2018.04.567","article-title":"Adjuvant chemotherapy for stage I ovarian clear cell carcinoma: Patterns of use and outcomes","volume":"150","author":"Nasioudis","year":"2018","journal-title":"Gynecol. Oncol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"60","DOI":"10.1093\/jnci\/djy071","article-title":"Invasive Epithelial Ovarian Cancer Survival by Histotype and Disease Stage","volume":"111","author":"Peres","year":"2019","journal-title":"J. Natl. Cancer Inst."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"1253","DOI":"10.1097\/AOG.0000000000003579","article-title":"Effects of Gastrointestinal-Type Chemotherapy in Women with Ovarian Mucinous Carcinoma","volume":"134","author":"Kurnit","year":"2019","journal-title":"Obstet. Gynecol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"541","DOI":"10.1016\/j.ygyno.2019.03.256","article-title":"An international, phase III randomized trial in patients with mucinous epithelial ovarian cancer (mEOC\/GOG 0241) with long-term follow-up: And experience of conducting a clinical trial in a rare gynecological tumor","volume":"153","author":"Gore","year":"2019","journal-title":"Gynecol. Oncol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"1256","DOI":"10.1056\/NEJMra1813254","article-title":"Mucinous Ovarian Carcinoma","volume":"380","author":"Morice","year":"2019","journal-title":"N. Engl. J. Med."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"231","DOI":"10.1111\/j.1365-2559.2005.02194.x","article-title":"Metastatic neoplasms involving the ovary: A review with an emphasis on morphological and immunohistochemical features","volume":"47","author":"McCluggage","year":"2005","journal-title":"Histopathology"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1007\/s00428-015-1771-2","article-title":"A national population-based study provides insight in the origin of malignancies metastatic to the ovary","volume":"467","author":"Bruls","year":"2015","journal-title":"Virchows Arch."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"1160","DOI":"10.1111\/IGC.0b013e3181b33cce","article-title":"Secondary ovarian malignancies: Frequency, origin, and characteristics","volume":"19","author":"Thomas","year":"2009","journal-title":"Int. J. Gynecol. Cancer"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"191","DOI":"10.1097\/PGP.0000000000000238","article-title":"Immunohistochemistry in the Diagnosis of Mucinous Neoplasms Involving the Ovary: The Added Value of SATB2 and Biomarker Discovery Through Protein Expression Database Mining","volume":"35","author":"Strickland","year":"2016","journal-title":"Int. J. Gynecol. Pathol."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"1071","DOI":"10.1016\/S1470-2045(11)70058-4","article-title":"Mucinous carcinomas of the ovary and colorectum: Different organ, same dilemma","volume":"12","author":"Kelemen","year":"2011","journal-title":"Lancet Oncol."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"1830","DOI":"10.1097\/PAS.0b013e3182299c25","article-title":"Determining the site of origin of mucinous adenocarcinoma: An immunohistochemical study of 175 cases","volume":"35","author":"Chu","year":"2011","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"208","DOI":"10.1093\/jjco\/hyp150","article-title":"CK7, CK20, CDX2 and MUC2 Immunohistochemical staining used to distinguish metastatic colorectal carcinoma involving ovary from primary ovarian mucinous adenocarcinoma","volume":"40","author":"Shin","year":"2010","journal-title":"Jpn. J. Clin. Oncol."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"376","DOI":"10.1002\/bjs5.50134","article-title":"Factors influencing long-term survival after cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for pseudomyxoma peritonei originating from appendiceal neoplasms","volume":"3","author":"Kok","year":"2019","journal-title":"BJS Open"},{"key":"ref_31","unstructured":"(2024, September 13). NCCN Guidelines Version 3.2024. Principles of Surgery. Available online: https:\/\/www.nccn.org\/guidelines\/guidelines-detail?category=1&id=1453."},{"key":"ref_32","unstructured":"Whitney, C.W., and Spirtos, N. (2010). Gynecologic Oncology Group Surgical Procedures Manual, Gynecologic Oncology Group."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"e32774","DOI":"10.1097\/MD.0000000000032774","article-title":"Neoadjuvant chemotherapy in advanced epithelial ovarian cancer by histology: A SEER based survival analysis","volume":"102","author":"Liu","year":"2023","journal-title":"Medicine"},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"2449","DOI":"10.1200\/JCO.2011.39.7166","article-title":"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","volume":"30","author":"Chua","year":"2012","journal-title":"J. Clin. Oncol."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"473","DOI":"10.1016\/j.ygyno.2021.09.023","article-title":"An open-label phase I dose-escalation study of the safety and pharmacokinetics of DMUC4064A in patients with platinum-resistant ovarian cancer","volume":"163","author":"Liu","year":"2021","journal-title":"Gynecol. Oncol."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"523","DOI":"10.1016\/j.ygyno.2019.12.024","article-title":"Front-line chemo-immunotherapy with carboplatin-paclitaxel using oregovomab indirect immunization in advanced ovarian cancer: A randomized phase II study","volume":"156","author":"Brewer","year":"2020","journal-title":"Gynecol. Oncol."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"100311","DOI":"10.1016\/j.esmoop.2021.100311","article-title":"Maintenance therapy of patients with recurrent epithelial ovarian carcinoma with the anti-tumor-associated-mucin-1 antibody gatipotuzumab: Results from a double-blind, placebo-controlled, randomized, phase II study","volume":"7","author":"Ledermann","year":"2022","journal-title":"ESMO Open."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"2289","DOI":"10.1245\/s10434-007-9462-0","article-title":"Critical analysis of treatment failure after complete cytoreductive surgery and perioperative intraperitoneal chemotherapy for peritoneal dissemination from appendiceal mucinous neoplasms","volume":"14","author":"Yan","year":"2007","journal-title":"Ann. Surg. Oncol."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1097\/00000658-199401000-00009","article-title":"The natural history of surgically treated primary adenocarcinoma of the appendix","volume":"219","author":"Nitecki","year":"1994","journal-title":"Ann. Surg."}],"container-title":["Life"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2075-1729\/14\/11\/1390\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T16:23:18Z","timestamp":1760113398000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2075-1729\/14\/11\/1390"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,10,29]]},"references-count":39,"journal-issue":{"issue":"11","published-online":{"date-parts":[[2024,11]]}},"alternative-id":["life14111390"],"URL":"https:\/\/doi.org\/10.3390\/life14111390","relation":{},"ISSN":["2075-1729"],"issn-type":[{"value":"2075-1729","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,10,29]]}}}