Pseudomyxoma Peritonei: Case Report and Literature Review

  • Keishla M. García
  • Karla M. Flores
  • Alejandro Ruiz
  • Frances L. González
  • Ángel M. Rodríguez
Case Report


Pseudomyxoma peritonei (PMP) is a disorder characterized by an intra-abdominal accumulation of mucin secondary to the growth of neoplastic mucin-secreting cells on peritoneal surfaces [1, 2]. PMP is a rare condition with an estimated incidence of one to two cases per million per year [3, 4]. Patients have an average age of 53 years at the time of diagnosis, being more commonly seen in females [5, 6]. In most cases, the neoplasm originates in the appendix and later spreads through the wall of the appendix into the peritoneal space [7, 8]. Reproduction and aggregation of free and implanted tumor cells lead to a progressive peritoneal mucinous tumor and ascites [9]. In other reported cases, the primary tumor origin has occasionally developed in other organs including the ovary, colorectum, gallbladder, stomach, pancreas, urachus, fallopian tube, lung, and breast [10, 11]. Initial presentation varies and consists of unspecified signs and symptoms that relate to the...



Pseudomyxoma peritonei


Disseminated peritoneal adenomucinosis


Peritoneal mucinous carcinomatosis


Cytoreduction surgery


Hyperthermic intraperitoneal chemotherapy


Carcinoembryonic antigen


National Comprehensive Cancer Network


Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no conflict of interest.


  1. 1.
    Misdraji J. Mucinous epithelial neoplasms of the appendix and pseudomyxoma peritonei. Mod Pathol. 2015;28(S1):S67–79.CrossRefGoogle Scholar
  2. 2.
    Carr NJ (2014). Current concepts in pseudomyxoma peritonei. In Annales de pathologie Elsevier Masson. (Vol. 34, No. 1, pp. 9-13)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.CrossRefGoogle Scholar
  4. 4.
    Bevan KE, Mohamed F, Moran BJ. Pseudomyxoma peritonei. World J Gastrointest Oncol. 2010;2(1):44–50.CrossRefGoogle 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.Google Scholar
  6. 6.
    Homeag M, Enachescu V, Pavel LE, Zaharia B, Manescu N. Pseudomyxoma peritonei. Curr Health Sci J. 2009;35(3):193–6.Google Scholar
  7. 7.
    Smeenk RM, Verwaal VJ, Zoetmulder FAN. Pseudomyxoma peritonei. Cancer Treat Rev. 2007;33(2):138–45.CrossRefGoogle Scholar
  8. 8.
    Ramaswamy V. Pathology of mucinous appendiceal tumors and pseudomyxoma peritonei. Indian J Surg Oncol. 2016;7(2):258–67.CrossRefGoogle Scholar
  9. 9.
    Sugarbaker PH. Pseudomyxoma peritonei. A cancer whose biology is characterized by a redistribution phenomenon. Ann Surg. 1994;219(2):109.CrossRefGoogle 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.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.Google Scholar
  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.CrossRefGoogle 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.CrossRefGoogle Scholar
  14. 14.
    Esquivel J, Sugarbaker PH. Clinical presentation of the pseudomyxoma peritonei syndrome. Br J Surg. 2000;87(10):1414–8.CrossRefGoogle 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.CrossRefGoogle 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.CrossRefGoogle 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.CrossRefGoogle 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.CrossRefGoogle Scholar
  19. 19.
    Sugarbaker PH. New standard of care for appendiceal epithelial neoplasms and pseudomyxoma peritonei syndrome? Lancet Oncol. 2006;7(1):69–76.CrossRefGoogle Scholar
  20. 20.
    Sugarbaker PH. Cytoreductive surgery and intraperitoneal chemotherapy with peritoneal spreadof cystadenocarcinoma. Eur J Surg Suppl. 1991;561:75–82.Google Scholar
  21. 21.
    Winder T, Lenz HJ. Mucinous adenocarcinomas with intra-abdominal dissemination: a review of current therapy. Oncologist. 2010;15(8):836–44.CrossRefGoogle 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.CrossRefGoogle Scholar
  23. 23.
    Kelly KJ. Management of appendix cancer. Clin Colon Rectal Surg. 2015;28(4):247–55.CrossRefGoogle 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.CrossRefGoogle 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.CrossRefGoogle 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.CrossRefGoogle 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.CrossRefGoogle Scholar
  28. 28.
    Morris DL. Peritonectomy HIPEC—contemporary results, indications. Chin J Cancer Res. 2013;25(4):373–4.Google Scholar
  29. 29.
    Sugarbaker PH. Peritonectomy procedures. Ann Surg. 1995;221(1):29–42.CrossRefGoogle 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.CrossRefGoogle Scholar
  31. 31.
    Zappa L, Savady R, Humphries GN, Sugarbaker PH. Interstitial pneumonitis following intrapleural chemotherapy. World J Surg Oncol. 2009;7(1):17.CrossRefGoogle 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.CrossRefGoogle 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.CrossRefGoogle 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.CrossRefGoogle 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.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.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.CrossRefGoogle Scholar
  38. 38.
    Kitai T. Pulmonary metastasis from pseudomyxoma peritonei. Gastroenterol Res Pract. 2012;2012:16.CrossRefGoogle Scholar
  39. 39.
    Sugarbaker PH, Rangole AK, Carr NJ. Peritoneal metastases from mucinous endocervical adenocarcinoma. Gynecol Oncol Reports. 2014;10:5–8.CrossRefGoogle 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.CrossRefGoogle 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.CrossRefGoogle 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.CrossRefGoogle 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.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.CrossRefGoogle 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.CrossRefGoogle 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.CrossRefGoogle 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.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. 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.Google Scholar
  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.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.CrossRefGoogle 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.CrossRefGoogle 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.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.CrossRefGoogle 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.CrossRefGoogle Scholar
  56. 56.
    Markman M. Intraperitoneal chemotherapy in the management of malignant disease. Expert Rev Anticancer Ther. 2001;1(1):142–8.CrossRefGoogle 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.CrossRefGoogle Scholar
  58. 58.
    Sugarbaker PH. Peritonectomy procedures. Surg Oncol Clin N Am. 2003;12(3):703–27.CrossRefGoogle 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. 101-111Google Scholar
  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.CrossRefGoogle 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.CrossRefGoogle 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.CrossRefGoogle 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.CrossRefGoogle 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.CrossRefGoogle Scholar
  66. 66.
    NCCN (National Comprehensive Cancer Network). Colon Canr: NCCN Clinical Practice Guidelines in Oncology (Version 4.2018). Accessed 29 July 2018.

Copyright information

© This is a U.S. government work and not under copyright protection in the U.S.; foreign copyright protection may apply 2019

Authors and Affiliations

  • Keishla M. García
    • 1
  • Karla M. Flores
    • 1
  • Alejandro Ruiz
    • 1
  • Frances L. González
    • 2
  • Ángel M. Rodríguez
    • 1
    • 3
  1. 1.Department of OncologySan Juan Bautista School of MedicineCaguasUSA
  2. 2.University of Puerto RicoSan JuanUSA
  3. 3.Surgical DepartmentSan Juan Bautista School of MedicineCaguasUSA

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