Pseudomyxoma Peritonei: Case Report and Literature Review

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Fig. 1



Pseudomyxoma peritonei


Disseminated peritoneal adenomucinosis


Peritoneal mucinous carcinomatosis


Cytoreduction surgery


Hyperthermic intraperitoneal chemotherapy


Carcinoembryonic antigen


National Comprehensive Cancer Network


  1. 1.

    Misdraji J. Mucinous epithelial neoplasms of the appendix and pseudomyxoma peritonei. Mod Pathol. 2015;28(S1):S67–79.

    PubMed  Google Scholar 

  2. 2.

    Carr NJ (2014). Current concepts in pseudomyxoma peritonei. In Annales de pathologie Elsevier Masson. (Vol. 34, No. 1, pp. 9-13)

    PubMed  Google Scholar 

  3. 3.

    Fernandes ACDO, Rocha GRMD, Oliveira ADD, Guimarães MD, Carvalho SCD, Chojniak R. Pseudomyxoma peritonei in a pediatric patient: a case report and literature review. Revista da Associação Médica Brasileira. 2018;64(2):195–9.

    PubMed  Google Scholar 

  4. 4.

    Bevan KE, Mohamed F, Moran BJ. Pseudomyxoma peritonei. World J Gastrointest Oncol. 2010;2(1):44–50.

    PubMed  PubMed Central  Google Scholar 

  5. 5.

    Chua TC, Moran BJ, Sugarbaker PH., Levine EA, Glehen O, Gilly FN, ... & Liauw W (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. J Clin Oncol, 30(20), 2449–2456.

  6. 6.

    Homeag M, Enachescu V, Pavel LE, Zaharia B, Manescu N. Pseudomyxoma peritonei. Curr Health Sci J. 2009;35(3):193–6.

    PubMed  PubMed Central  Google Scholar 

  7. 7.

    Smeenk RM, Verwaal VJ, Zoetmulder FAN. Pseudomyxoma peritonei. Cancer Treat Rev. 2007;33(2):138–45.

    CAS  PubMed  Google Scholar 

  8. 8.

    Ramaswamy V. Pathology of mucinous appendiceal tumors and pseudomyxoma peritonei. Indian J Surg Oncol. 2016;7(2):258–67.

    PubMed  PubMed Central  Google Scholar 

  9. 9.

    Sugarbaker PH. Pseudomyxoma peritonei. A cancer whose biology is characterized by a redistribution phenomenon. Ann Surg. 1994;219(2):109.

    CAS  PubMed  PubMed Central  Google Scholar 

  10. 10.

    Prayson RA, Hart WR, Petras RE. Pseudomyxoma peritonei. A clinicopathologic study of 19 cases with emphasis on site of origin and nature of associated ovarian tumors. Am J Surg Pathol. 1994;18(6):591–603.

    CAS  PubMed  Google Scholar 

  11. 11.

    Agrawal AK, Bobiński P, Grzebieniak Z, Rudnicki J, Marek G, Kobielak P, ... & Hałoń A (2014). Pseudomyxoma peritonei originating from urachus—case report and review of the literature. Curr Oncol, 21(1), e155.

  12. 12.

    Nawaz A, Karakurum A, Weltman D, Shehata A, Mohammed I, Hadjiyane C, et al. Pseudomyxoma peritonei manifesting as intestinal obstruction. South Med J. 2000;93(9):891–3.

    CAS  PubMed  Google Scholar 

  13. 13.

    Touloumis Z, Galyfos G, Kavouras N, Menis M, Lavant L. Aggressive pseudomyxoma peritonei: a case report with an unusual clinical presentation. Case Reports Oncol Med. 2013;2013:1–4.

    Google Scholar 

  14. 14.

    Esquivel J, Sugarbaker PH. Clinical presentation of the pseudomyxoma peritonei syndrome. Br J Surg. 2000;87(10):1414–8.

    CAS  PubMed  Google Scholar 

  15. 15.

    Noguchi R, Yano H, Gohda Y, Suda R, Igari T, Ohta Y, et al. Molecular profiles of high-grade and low-grade pseudomyxoma peritonei. Cancer Med. 2015;4(12):1809–16.

    CAS  PubMed  PubMed Central  Google Scholar 

  16. 16.

    Amini A, Masoumi-Moghaddam S, Ehteda A, Morris DL. Secreted mucins in pseudomyxoma peritonei: pathophysiological significance and potential therapeutic prospects. Orphanet J Rare Dis. 2014;9(1):71.

    PubMed  PubMed Central  Google Scholar 

  17. 17.

    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 “pseudomyxoma peritonei”. Am J Surg Pathol. 1995;19(12):1390–408.

    CAS  PubMed  PubMed Central  Google Scholar 

  18. 18.

    Bradley RF, Stewart JH IV, 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(5):551–9.

    PubMed  PubMed Central  Google Scholar 

  19. 19.

    Sugarbaker PH. New standard of care for appendiceal epithelial neoplasms and pseudomyxoma peritonei syndrome? Lancet Oncol. 2006;7(1):69–76.

    PubMed  Google Scholar 

  20. 20.

    Sugarbaker PH. Cytoreductive surgery and intraperitoneal chemotherapy with peritoneal spreadof cystadenocarcinoma. Eur J Surg Suppl. 1991;561:75–82.

  21. 21.

    Winder T, Lenz HJ. Mucinous adenocarcinomas with intra-abdominal dissemination: a review of current therapy. Oncologist. 2010;15(8):836–44.

    PubMed  PubMed Central  Google Scholar 

  22. 22.

    Li C, Kanthan R, Kanthan SC. Pseudomyxoma peritonei–a revisit: report of 2 cases and literature review. World J Surg Oncol. 2006;4(1):60.

    PubMed  PubMed Central  Google Scholar 

  23. 23.

    Kelly KJ. Management of appendix cancer. Clin Colon Rectal Surg. 2015;28(4):247–55.

    PubMed  PubMed Central  Google Scholar 

  24. 24.

    Tentes AA, Tripsiannis G, Markakidis SK, Karanikiotis CN, Tzegas G, Georgiadis G, et al. Peritoneal cancer index: a prognostic indicator of survival in advanced ovarian cancer. Eur J Surg Oncol (EJSO). 2003;29(1):69–73.

    Google Scholar 

  25. 25.

    Heaney RM, Shields C, Mulsow J. Outcome following incomplete surgical cytoreduction combined with intraperitoneal chemotherapy for colorectal peritoneal metastases. World J Gastrointest Oncol. 2015;7(12):445–54.

    PubMed  PubMed Central  Google Scholar 

  26. 26.

    Di Giorgio A, Naticchioni E, Biacchi D, Sibio S, Accarpio F, Rocco M, et al. Cytoreductive surgery (peritonectomy procedures) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) in the treatment of diffuse peritoneal carcinomatosis from ovarian cancer. Cancer. 2008;113(2):315–25.

    PubMed  Google Scholar 

  27. 27.

    Gilly FN, Cotte E, Brigand C, Monneuse O, Beaujard AC, Freyer G, et al. Quantitative prognostic indices in peritoneal carcinomatosis. Eur J Surg Oncol (EJSO). 2006;32(6):597–601.

    CAS  Google Scholar 

  28. 28.

    Morris DL. Peritonectomy HIPEC—contemporary results, indications. Chin J Cancer Res. 2013;25(4):373–4.

    PubMed  PubMed Central  Google Scholar 

  29. 29.

    Sugarbaker PH. Peritonectomy procedures. Ann Surg. 1995;221(1):29–42.

    CAS  PubMed  PubMed Central  Google Scholar 

  30. 30.

    Haase E, Yoo D, Sugarbaker PH. Management of appendiceal pseudomyxoma peritonei diagnosed during pregnancy. World J Surg Oncol. 2009;7(1):48.

    PubMed  PubMed Central  Google Scholar 

  31. 31.

    Zappa L, Savady R, Humphries GN, Sugarbaker PH. Interstitial pneumonitis following intrapleural chemotherapy. World J Surg Oncol. 2009;7(1):17.

    PubMed  PubMed Central  Google Scholar 

  32. 32.

    Khan AB, Al Suhaibani Y, Al Mohaimed K, Morris DL. Application of advanced multimodality care to pseudomyxoma peritonei patient: report of first patient treated at a tertiary center. Indian J Surg Oncol. 2010;1(3):270–3.

    PubMed  PubMed Central  Google Scholar 

  33. 33.

    El-Safadi S, Stahl U, Tinneberg HR, Hackethal A, Muenstedt K. Primary signet ring cell mucinous ovarian carcinoma: a case report and literature review. Case Reports Oncol. 2010;3(3):451–7.

    Google Scholar 

  34. 34.

    Fonseca LMD, Lacerda-Filho A, Silva RGD. Pseudomyxoma peritonei syndrome 12 months after an intact resection of malignant mucocele of the appendix: a case report. Clinics. 2010;65(8):817–8.

    PubMed  PubMed Central  Google Scholar 

  35. 35.

    Morris-Stiff G, Falk GA, Joyce D, Chalikonda S. Unusual presentation of more common disease/injury: unusual contents of a scrotal swelling. BMJ Case Reports. 2011;2011:bcr0320114023.

    PubMed  PubMed Central  Google Scholar 

  36. 36.

    Bouchereau M, Gervais MK, Sideris L, Loriot MH, Ahern SP, Dubé P. Hepatic necrosis and hemorrhage following hyperthermic intraperitoneal chemotherapy with oxaliplatin: a review of two cases. J Gastrointest Oncol. 2011;2(2):113–6.

    PubMed  PubMed Central  Google Scholar 

  37. 37.

    Laskov I, Michaan N, Many A, Amit A, Azem F. Successful pregnancy following transfer of frozen-thawed embryos in a patient with pseudomyxoma peritonei who underwent peritonectomy and bilateral oophorectomy. J Gynecol Oncol. 2012;23(2):129–32.

    PubMed  PubMed Central  Google Scholar 

  38. 38.

    Kitai T. Pulmonary metastasis from pseudomyxoma peritonei. Gastroenterol Res Pract. 2012;2012:16.

    Google Scholar 

  39. 39.

    Sugarbaker PH, Rangole AK, Carr NJ. Peritoneal metastases from mucinous endocervical adenocarcinoma. Gynecol Oncol Reports. 2014;10:5–8.

    Google Scholar 

  40. 40.

    Singh VK, Nishant K, Sharma BK, Lamichaney R. Case report: a weeping umbilical hernia: bilateral ovarian mucinous cystadenoma with disseminated peritoneal adenomucinosis. BMJ Case Reports. 2014;2014:bcr2014204748.

    PubMed  PubMed Central  Google Scholar 

  41. 41.

    Murughan K, Tiwari M, Balakrishnan K. Cytoreduction with hyperthermic intraperitoneal chemotherapy: an anaesthetic challenge. Indian J Anaesth. 2014;58(1):59–62.

    PubMed  PubMed Central  Google Scholar 

  42. 42.

    Tempfer CB, Solass W, Buerkle B, Reymond MA. Pressurized intraperitoneal aerosol chemotherapy (PIPAC) with cisplatin and doxorubicin in a woman with pseudomyxoma peritonei: a case report. Gynecol Oncol Reports. 2014;10:32–5.

    Google Scholar 

  43. 43.

    Peixoto RDA, Wilson S, Schaeffer DF, Lim HJ. Pseudomyxoma peritonei metastatic to the bone: case report and review of systemic management. Gastrointest Cancer Res: GCR. 2014;7(3–4):108.

    PubMed  Google Scholar 

  44. 44.

    Campanati RG, Hanan B, Souza SSD, Gomes da Silva R. Pregnancy after cytoreductive surgery and hyperthermic intraperitoneal chemotherapy in pseudomyxoma peritonei. Case Reports Surg. 2014;2014:13.

    Google Scholar 

  45. 45.

    Sabbagh C, Vaillandet C, Tuech JJ, Regimbeau JM. A new etiology for the abdominal compartment syndrome: pseudomyxoma peritonei. Case Reports Gastroenterol. 2015;9(3):307–10.

    Google Scholar 

  46. 46.

    Chauhan A, Patodi N, Ahmed M. A rare cause of ascites: pseudomyxoma peritonei and a review of the literature. Clin Case Reports. 2015;3(3):156–9.

    Google Scholar 

  47. 47.

    Anania G, Giaccari S, Solfrini G, Scagliarini L, Vedana L, Resta G. Appendicular mucocele: two case reports and literature review. Il Giornale di chirurgia. 2015;36(6):276–9.

    CAS  PubMed  Google Scholar 

  48. 48.

    Chiruvella A, Staley CA, Khanna N, Russell M, Maithel SK, et al. Pseudomyxoma Peritonei from a borderline mucinous tumor arising in an ovarian mature cystic teratoma: a rare case report. Arch Surg Oncol. 2016;2:114.

    Article  Google Scholar 

  49. 49.

    Gohda Y, Noguchi R, Horie T, Igari T, Nakamura H, Ohta Y, ... & Furukawa Y (2016). Pseudomyxoma peritonei of a mature ovarian teratoma caused by mismatch repair deficiency in a patient with Lynch syndrome: a case report. BMC Med Gen, 17(1), 94.

  50. 50.

    Ababneh R, Piso P, Hofmann HS, Dumitrovici A, Buiga R, Samasca G, et al. An invasive treatment of pseudomyxoma peritonei with intrathoracic involvement. Maedica. 2016;11(1):72–5.

    PubMed  PubMed Central  Google Scholar 

  51. 51.

    Mathur S, Kelly E. Pseudomyxoma peritonei: a case report of an incidental finding at cesarean section. J Clin Gynecol Obstet. 2016;5(1):32–6.

    Google Scholar 

  52. 52.

    Vavinskaya V, Baumgartner JM, Ko A, Saenz CC, Valasek MA. Low-grade appendiceal mucinous neoplasm involving the endometrium and presenting with mucinous vaginal discharge. Case Rep Obstet Gynecol. 2016;2016:14.

    Google Scholar 

  53. 53.

    McGrath C, Linden K, Hube P, Adamiak A, Dennis K. Palliative radiation therapy for symptom control in an advanced case of pseudomyxoma peritonei. Cureus. 2017;9(6):e1407.

    PubMed  PubMed Central  Google Scholar 

  54. 54.

    Liang L, Zhou N, Xu H, Liu D, Lu Y, Li F, et al. Urachal mucinous adenocarcinoma with pseudomyxoma peritonei: a case report. Medicine. 2017;96(35):e7548.

    PubMed  PubMed Central  Google Scholar 

  55. 55.

    González-Moreno S, González-Bayón LA, Ortega-Pérez G. Hyperthermic intraperitoneal chemotherapy: rationale and technique. World J Gastrointest Oncol. 2010;2(2):68–75.

    PubMed  PubMed Central  Google Scholar 

  56. 56.

    Markman M. Intraperitoneal chemotherapy in the management of malignant disease. Expert Rev Anticancer Ther. 2001;1(1):142–8.

    CAS  PubMed  Google Scholar 

  57. 57.

    Loungnarath R, Causeret S, Bossard N, Faheez M, Sayag-Beaujard AC, Brigand C, et al. Cytoreductive surgery with intraperitoneal chemohyperthermia for the treatment of pseudomyxoma peritonei: a prospective study. Dis Colon Rectum. 2005;48(7):1372–9.

    PubMed  Google Scholar 

  58. 58.

    Sugarbaker PH. Peritonectomy procedures. Surg Oncol Clin N Am. 2003;12(3):703–27.

    PubMed  Google Scholar 

  59. 59.

    Sugarbaker PH, Gianola FJ, Speyer JL, Wesley R, Barofsky I, & Myers CE (1985). Prospective randomized trial of intravenous v intraperitoneal 5-FU in patients with advanced primary colon or rectal cancer. In Seminars in oncology. Elsevier. Vol. 12, no. 3, pp. 101-111

  60. 60.

    Ortega-Deballon P, Glehen O, Levine E, Piso P, Sugarbaker PH, Hayes-Jordan A, et al. Childbearing after hyperthermic intraperitoneal chemotherapy: results from an international survey. Ann Surg Oncol. 2011;18(8):2297–301.

    PubMed  PubMed Central  Google Scholar 

  61. 61.

    Glehen O, Kwiatkowski F, Sugarbaker PH, et al. Cytoreductive surgery combined with perioperative intraperitoneal chemotherapy for the management of peritoneal carcinomatosis from colorectal cancer: a multi-institutional study. J Clin Oncol. 2004;22:3284–92.

    CAS  PubMed  Google Scholar 

  62. 62.

    Sugarbaker PH, Chang D. Results of treatment of 385 patients with peritoneal surface spread of appendiceal malignancy. Ann Surg Oncol. 1999;6(8):727–31.

    CAS  PubMed  PubMed Central  Google Scholar 

  63. 63.

    Sugarbaker PH. Managing the peritoneal surface component of gastrointestinal cancer. Part 2. Perioperative intraperitoneal chemotherapy. Oncology (Williston Park, NY). 2004;18(2):207–19.

    Google Scholar 

  64. 64.

    Pablo Carmignani C, Hampton R, Sugarbaker CE, Chang D, Sugarbaker PH. Utility of CEA and CA 19-9 tumor markers in diagnosis and prognostic assessment of mucinous epithelial cancers of the appendix. J Surg Oncol. 2004;87(4):162–6.

    PubMed  Google Scholar 

  65. 65.

    Van Ruth S, Hart AAM, Bonfrer JMG, Verwaal VJ, Zoetmulder FAN. Prognostic value of baseline and serial carcinoembryonic antigen and carbohydrate antigen 19.9 measurements in patients with pseudomyxoma peritonei treated with cytoreduction and hyperthermic intraperitoneal chemotherapy. Ann Surg Oncol. 2002;9(10):961–7.

    PubMed  Google Scholar 

  66. 66.

    NCCN (National Comprehensive Cancer Network). Colon Canr: NCCN Clinical Practice Guidelines in Oncology (Version 4.2018). Accessed 29 July 2018.

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Correspondence to Ángel M. Rodríguez.

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García, K.M., Flores, K.M., Ruiz, A. et al. Pseudomyxoma Peritonei: Case Report and Literature Review. J Gastrointest Canc 50, 1037–1042 (2019).

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