Skip to main content

Pseudomyxoma Peritonei

  • Chapter
  • 895 Accesses

Part of the book series: Updates in Surgery ((UPDATESSURG))

Abstract

Pseudomyxoma peritonei (PMP) is a rare, low-grade, peritoneal malignancy predominated by mucus, with an estimated incidence of two to three cases per million people per year [1]. Werth, in 1884, first described PMP as a rare, unusual reaction of the peritoneum to a jelly-like substance, looking like a myxoma, in relation to an ovarian neoplasm [2]. In 1901, Frankel described PMP associated with an appendiceal cyst [3], but the first description of a benign mucocele of the appendix was reported by Rokitansky in 1842 [4].

This is a preview of subscription content, log in via an institution.

Buying options

Chapter
USD   29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD   84.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD   109.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Learn about institutional subscriptions

References

  1. Smeenk RM, van Velthuysen ML, Verwaal VJ et al (2008) Appendiceal neoplasms and pseudomyxoma peritonei: a population based study. Eur J Surg Oncol 34:196–201

    Article  CAS  PubMed  Google Scholar 

  2. Werth R (1884) Klinische und anatomische untersuchungen zur lehre von den bauchgeschwuelsten und der laparotomie. Arch Gynaecol Obstet 24:100–118

    Google Scholar 

  3. Fraenkel E (1901) Ueber das sogennante pseudomyxoma peritonei. Munch Med Wochenschr 48:965–971

    Google Scholar 

  4. Weaver CH (1937) Mucocele of the appendix with pseudomucinous degeneration. Am J Surg 36:523

    Article  Google Scholar 

  5. Sugarbaker PH, Ronnett BM, Archer A et al (1996) Pseudomyxoma peritonei syndrome. Adv Surg 30:233–280

    CAS  PubMed  Google Scholar 

  6. Cheng KK (1949) An experimental study of mucocele of the appendix and pseudomyxoma peritonei. J Pathol Bacteriol 61:217–225

    Article  CAS  PubMed  Google Scholar 

  7. Grodinsky M, Rubnitz AS (1941) Mucocele of the appendix and pseudomyxoma peritonei: a clinical review and experimental study, with case report. Surg Gynecol Obstet 73:345–354

    Google Scholar 

  8. Baratti D, Kusamura S, Nonaka D et al (2008) Pseudomyxoma peritonei: clinical pathological and biological prognostic factors in patients treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC). Ann Surg Oncol 15:526–534

    Article  PubMed  Google Scholar 

  9. Jarvinen P, Lepisto A (2010) Clinical presentation of pseudomyxoma peritonei. Scand J Surg 99:213–216

    CAS  PubMed  Google Scholar 

  10. Carr NJ, Emory TS, Sobin LH (2002) Epithelial neoplasms of the appendix and colorectum: an analysis of cell proliferation, apaptosis and expression of p53, CD44, bcl-2. Arch Pathol Lab Med 126:837–841

    CAS  PubMed  Google Scholar 

  11. Szych C, Staebler A, Connolly DC et al (1999) Molecular genetic evidence supporting the clonality and appendiceal origin of pseudomyxoma peritonei in women. Am J Pathol 154:1849–1855

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  12. Ronnett BM, Zahn CM, Kurman RJ et al (1995) 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 19:1390–1408

    Article  CAS  PubMed  Google Scholar 

  13. Mukherjee A, Parvaiz A, Cecil TD et al (2004) Pseudomyxoma peritonei usually originates from the appendix: a review of the evidence. Eur J Gynaecol Oncol 25:411–414

    CAS  PubMed  Google Scholar 

  14. Young RH, Gilks CB, Scully RE (1991) Mucinous tumors of the appendix associated with mucinous tumors of the ovary and pseudomyxoma peritonei. A clinicopathological analysis of 22 cases supporting an origin in the appendix. Am J Surg Pathol 15:415–429

    Article  CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  16. Ronnett BM, Shmookler BM, Diener-West M et al (1997) Immunohistochemical evidence supporting the appendiceal origin of pseudomyxoma peritonei in women. Int J Gynecol Pathol 16:1–9

    Article  CAS  PubMed  Google Scholar 

  17. Guerrieri C, Franlund B, Fristedt S et al (1997) Mucinous tumors of the vermiform appendix and ovary, and pseudomyxoma peritonei: histogenetic implications of cytokeratin 7 expression. Hum Pathol 28:1039–1045

    Article  CAS  PubMed  Google Scholar 

  18. Baratti D, Kusamura S, Nonaka D et al (2009) Pseudomyxoma Peritonei: Biological Features Are the Dominant Prognostic Determinants After Complete Cytoreduction and HyperthermicIntraperitoneal Chemotherapy. Ann Surg 249:243–249

    Article  PubMed  Google Scholar 

  19. Ronnett BM, Seidman JD (2003) Mucinous tumors arising in ovarian mature cystic teratomas: relationship to the clinical syndrome of pseudomyxoma peritonei. Am J Surg Pathol 27:650–657

    Article  PubMed  Google Scholar 

  20. McKenney JK, Soslow RA, Longacre TA (2008) Ovarian mature teratomas with mucinous epithelial neoplasms: morphologic heterogeneity and association with pseudomyxoma peritonei. Am J Surg Pathol 32:645–655

    Article  PubMed  Google Scholar 

  21. Sugarbaker PH (1996) Pseudomyxoma peritonei. Cancer Treat Res 81:105–119

    Article  CAS  PubMed  Google Scholar 

  22. Misdraji J, Yantiss RK, Graeme-Cook FM et al (2003) Appendiceal mucinous neoplasms: a clinicopathologic analysis of 107 cases. Am J Surg Pathol 27:1089–1103

    Article  PubMed  Google Scholar 

  23. Renehan A, O’Dwyer S, Stern P (2009) Pseudomyxoma peritonei. In: Schwab M (ed) Cancer encyclopedia. Springer, London, pp 2134–2138

    Google Scholar 

  24. Panarelli NC, Yantiss RK (2011) Mucinous neoplasms of the appendix and peritoneum. Arch Pathol Lab Med 135:1261–1268

    Article  PubMed  Google Scholar 

  25. Bosman FT, Carneiro F, Hruban RH et al (2010) Classification of tumours of the digestive system. 4th ed. Lyon, France: IARC:112–125

    Google Scholar 

  26. Bradley RF, Stewart JH, Russell GB et al (2006) Pseudomyxoma peritonei of appendiceal origin: a clinicopathologic analysis of 101 patients uniformly treated at a single institution, with literature review. Am J Surg Pathol 30:551–559

    Article  PubMed  Google Scholar 

  27. Van ES, Offerhaus GJ, Hart AA et al (2007) Goblet cell carcinoid of the appendix: a specific type of carcinoma. Histopathology 51:763–773

    Article  Google Scholar 

  28. Sugarbaker PH (1994) Pseudomyxoma peritonei. A cancer whose biology is characterized by a redistribution phenomenon. Ann Surg 219:109–111

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  29. Young RH (2004) Pseudomyxoma peritonei and selected other aspects of the spread of appendiceal neoplasms. Semin Diagn Pathol 21:134–150

    Article  PubMed  Google Scholar 

  30. Lee BY, Kim HS, Lee SH et al (2004) Pseudomyxoma peritonei: extraperitoneal spread to the pleural cavity and lung. J Thorac Imaging 19:123–126

    Article  PubMed  Google Scholar 

  31. Mortman KD, Sugarbaker PA, Shmookler BM et al (1997) Pulmonary metastases in pseudomyxoma peritonei syndrome. Ann Thorac Surg 64:1434–1436

    Article  CAS  PubMed  Google Scholar 

  32. Geisinger KR, Levine EA, Shen P et al (2007) Pleuropulmonary involvement in pseudomyxoma peritonei: morphologic assessment and literature review. Am J Clin Pathol 127:135–143

    Article  PubMed  Google Scholar 

  33. Peek DF, Beets GL (1999) Pseudomyxoma peritonei in the pleural cavity: report of a case. Dis Colon Rectum 42:113–115

    Article  CAS  PubMed  Google Scholar 

  34. Yoshida R, Yoshioka K, Yoshitaka H et al (2002) Pseudomyxoma peritonei of appendiceal cancer with metastasis to the stomach: report of a case. Surg Today 32:547–550

    Article  PubMed  Google Scholar 

  35. Smeenk RM, Verwaal VJ, Antonini N et al (2007) Progression of pseudomyxoma peritonei after combined modality treatment: management and outcome. Ann Surg Oncol 14:493–499

    Article  PubMed  Google Scholar 

  36. Mets T, Van Hove W, Louis H (1977) Pseudomyxoma peritonei. Report of a case with extraperitoneal metastasis and invasion of the spleen. Chest 72:792–794

    Article  CAS  PubMed  Google Scholar 

  37. Smeenk RM, Verwaal VJ, Zoetmulder FA (2007) Pseudomyxoma peritonei. Cancer Treat Rev 33:138–145

    Article  CAS  PubMed  Google Scholar 

  38. Kusamura S, Hutanu I, Baratti D et al (2013) Circulating tumor markers: predictors of incomplete cytoreduction and powerful determinants of outcome in pseudomyxoma peritonei. J Surg Oncol 108:1–8

    Article  CAS  PubMed  Google Scholar 

  39. Koh JL, Liauw W, Chua T et al (2013) Carbohydrate antigen 19–9 (CA 19–9) is an independent prognostic indicator in pseudomyxoma peritonei post cytoreductive surgery and perioperative intraperitoneal chemotherapy. J Gastrointest Oncol 4:173–181

    PubMed Central  CAS  PubMed  Google Scholar 

  40. Taflampas P, Dayal S, Chandrakumaran K et al (2014) Pre-operative tumour marker status predicts recurrence and survival after complete cytoreduction and hyperthermic intraperitoneal chemotherapy for appendiceal Pseudomyxoma Peritonei: Analysis of 519 patients. Eur J Surg Oncol http://dx.doi.org/10.1016/j.ejso.2013.12.021.

    Google Scholar 

  41. Baratti D, Kusamura S, Martinetti A, et al (2007) Prognostic value of circulating tumor markers in patients with pseudomyxoma peritonei treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. Ann Surg Oncol 14:2300–2308

    Article  PubMed  Google Scholar 

  42. Alexander-Sefre F, Chandrakumaran K, Banerjee S et al (2005) Elevated tumour markers prior to complete tumour removal in patients with pseudomyxoma peritonei predict early recurrence. Colorectal Dis 7:382–386

    Article  CAS  PubMed  Google Scholar 

  43. Gollub MJ, DeCorato D, Schwartz LH (2000) MR enteroclysis: evaluation of small-bowel obstruction in a patient with pseudomyxoma peritonei. Am J Roentgenol 174:688–690.

    Article  CAS  Google Scholar 

  44. Passot G, Glehen O, Pellet O (2010) Pseudomyxoma peritonei: role of 18F-FDG PET in preoperative evaluation of pathological grade and potential for complete cytoreduction. Eur J Surg Oncol 36:315–323

    Article  CAS  PubMed  Google Scholar 

  45. Jarvinen P, Ristimaki A, Kantonen J et al (2013) Feasibility of radical cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for pseudomyxoma peritonei of appendiceal origin. Scand J Surg 102:145–151

    Article  CAS  PubMed  Google Scholar 

  46. Andreasson H, Graf W, Nygren P et al (2012) Outcome differences between debulking surgery and cytoreductive surgery in patients with pseudomyxoma peritonei. Eur J Surg Oncol 38:962–968

    Article  CAS  PubMed  Google Scholar 

  47. Jarvinen P, Jarvinen HJ, Lepisto A (2010) Survival of patients with pseudomyxoma peritonei treated by serial debulking. Colorectal Dis 12:868–872

    Article  CAS  PubMed  Google Scholar 

  48. Gough DB, Bonohue JH, Schutt AJ et al (1994) Pseudomyxoma peritonei. Long-term patient survival with an aggressive regional approach. Ann Surg 219:112–119

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  49. Miner TJ, Shia J, Jaques DP et al (2005) Long-term survival following treatment of pseudomyxoma peritonei: an analysis of surgical therapy. Ann Surg 241:300–308

    Article  PubMed Central  PubMed  Google Scholar 

  50. Nitecki SS, Wolff BG, Schlinkert R et al (1994) The natural history of surgically treated primary adenocarcinoma of the appendix. Ann Surg 219:51–57

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  51. Sugarbaker PH (2001) Cytoreductive surgery and perioperative intraperitoneal chemotherapy as a curative approach to pseudomyxoma peritonei syndrome. Eur J Surg Oncol 27:239–243

    Article  CAS  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  53. Moran B, Baratti D, Yan TD et al (2008) Consensus statement on the loco-regional treatment of appendiceal mucinous neoplasms with peritoneal dissemination (Pseudomyxoma peritonei). J Surg Oncol 98:277–282

    Article  PubMed  Google Scholar 

  54. Yan TD, Black D, Savady R et al (2006) A systematic review on the efficacy of cytoreductive surgery and perioperative intraperitoneal chemotherapy for pseudomyxoma peritonei. Ann Surg Oncol 14:484–492

    Article  PubMed  Google Scholar 

  55. Esquivel J, Sugarbaker PH (2001) Second-look surgery in patients with peritoneal dissemination from appendiceal malignancy: analysis of prognostic factors in 98 patients. Ann Surg 234(2):198–205

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  56. Sugarbaker PH (1999) Management of peritoneal-surface malignancy: the surgeon’s role. Langenbecks Arch Surg 384:576–587

    Article  CAS  PubMed  Google Scholar 

  57. De Simone M, Barone R, Vaira M et al (2003) Semi-closed hyperthermic-antiblastic peritoneal perfusion (HAPP) in the treatment of peritoneal carcinosis. J Surg Oncol 82:138–140

    Article  PubMed  Google Scholar 

  58. Glehen O, Sugarbaker PH, Elias D et al (2004) Cytoreductive surgery combined with perioperative intraperitoneal chemotherapy for the management of peritoneal carcinomatosis from colorectal cancer. A multi-institutional study of 506 patients. J Clin Oncol 22:3284–3292

    Article  CAS  PubMed  Google Scholar 

  59. McBride K, McFadden D, Osler T (2013) Improved survival of patients with pseudomyxoma peritonei receiving intraperitoneal chemotherapy with cytoreductive surgery: a systematic review and meta-analysis. J Surg Res 183:246–252

    Article  PubMed  Google Scholar 

  60. Robella M, Vaira M, Marsanic P et al (2013) Treatment of pseudomyxoma peritonei with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC): a single center experience. Minerva Chir 68:569–577

    CAS  PubMed  Google Scholar 

  61. Arjona-Sanchez A, Munoz-Casares FC, Casado-Adam A et al (2013) Outcome of patients with aggressive pseudomyxoma peritonei treated by cytoreductive surgery and intraperitoneal chemotherapy. World J Surg 37:1263–1270

    Article  PubMed  Google Scholar 

  62. Chua TC, Moran BJ, Sugarbaker PH et al (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 30:2449–2456

    Article  PubMed  Google Scholar 

  63. Sorensen O, Flatmark K, Reed W et al (2012) Evaluation of complete cytoreductive surgery and two intraperitoneal chemotherapy techniques in pseudomyxoma peritonei. Eur J Surg Oncol 38:969–976

    Article  CAS  PubMed  Google Scholar 

  64. Chua TC, Liauw W, Morris DL (2011) Early recurrence of pseudomyxoma peritonei following treatment failure of cytoreductive surgery and perioperative intraperitoneal chemotherapy is indicative of a poor survival outcome. Int J Colorectal Dis 27:381–389

    Article  PubMed  Google Scholar 

  65. Youssef H, Newman C, Chandrakumaran K et al (2011) Operative findings, early complications, and long-term survival in 456 patients with pseudomyxoma peritonei syndrome of appendiceal origin. Dis Colon Rectum 54:293–299

    Article  PubMed  Google Scholar 

  66. Elias D, Gilly F, Quenet F et al (2010) Pseudomyxoma peritonei: A French multicentric study of 301 patients treated with cytoreductive surgery and intraperitoneal chemotherapy. Eur J Surg Oncol 36:456–462

    Article  CAS  PubMed  Google Scholar 

  67. Alves S, Mohamed F, Yadegarfar G et al (2010) Prospective longitudinal study of quality of life following cytoreductive surgery and intraperitoneal chemotherapy for pseudomyxoma peritonei. Eur J Surg Oncol 36:1156–1161

    Article  CAS  PubMed  Google Scholar 

  68. Vaira M, Cioppa T, De Marco G et al (2009) Management of pseudomyxoma peritonei by cytoreduction + HIPEC (Hyperthermic Intraperitoneal Chemotherapy): results analysis of a twelve-year experience. In Vivo 23:639–644

    PubMed  Google Scholar 

  69. Elias D, Honoré C, Ciuchende R et al (2008) Peritoneal pseudomyxoma: results of systematic policy of complete cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. Br J Surg 95:1164–1171

    Article  CAS  PubMed  Google Scholar 

  70. Smeenk RM, Verwaal VJ, Antonini N et al (2007) Survival analysis of pseudomyxoma peritonei patients treated by cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. Ann Surg 245:104–109

    Article  PubMed Central  PubMed  Google Scholar 

  71. Gonzalez-Moreno S, Sugarbaker PH (2004) Right hemicolectomy does not confer a survival advantage in patients with mucinous carcinoma of the appendix and peritoneal seeding. Br J Surg 91:304–311

    Article  CAS  PubMed  Google Scholar 

  72. Sugarbaker PH, Alderman R, Edwards G et al (2006) Prospective morbidity and mortality assessment of cytoreductive surgery plus perioperative intraperitoneal chemotherapy to treat peritoneal dissemination of appendiceal mucinous malignancy. Ann Surg Oncol 13:1–11

    Article  Google Scholar 

  73. Smeenk RM, Verwaal VJ, Zoetmulder FAN (2006) Toxicity and mortality of cytoreduction and intraoperative hyperthermic intraperitoneal chemotherapy in pseudomyxoma peritonei— a report of 103 procedures. Eur J Surg Oncol 32:186–190

    Article  CAS  PubMed  Google Scholar 

  74. Deraco M, De Simone M, Rossi CR et al (2003) An Italian multicentric phase II study on peritonectomy and intra-peritoneal hyperthermic perfusion (IPHP) to treat patients with pseudomyxoma peritonei. J Exp Clin Cancer Res 22:35–39

    CAS  PubMed  Google Scholar 

  75. Berkovic P, van de Voorde L, De Meerleer G et al (2014) Whole abdominopelvic radiotherapy in the palliative treatment of pseudomyxoma peritonei. Strahlenther Onkol 190:223–228

    Article  CAS  PubMed  Google Scholar 

  76. Pai Rk, Beck AH, Norton JA et al (2009) Appendiceal mucinous neoplasms: clinicopathologic study of 116 cases with analysis of factors predicting recurrence. Am J Surg Pathol 33:1425–1439

    Article  PubMed  Google Scholar 

  77. Smeenk RM, van Velthuysen ML, Verwaal VJ et al (2008) Appendiceal neoplasms and pseudomyxoma peritonei: a population based study. Eur J Surg Oncol 34:196–201

    Article  CAS  PubMed  Google Scholar 

  78. McDonald JR, O’Dwyer ST, Rout S et al (2012) Classification of cytoreductive surgery for low grade appendiceal mucinous neoplasms. Br J Surg 99:987–992

    Article  CAS  PubMed  Google Scholar 

  79. Fish R, Selvasekar C, Crichton P et al (2014) Risk-reducing laparoscopic cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for low-grade appendiceal mucinous neoplasm: early outcomes and technique. Surg Endosc 25:341–345

    Article  Google Scholar 

  80. Dhage-Ivatury S, Shugarbaker PH (2006) Update on the surgical approach to mucocele of the appendix. J Am Coll Surg 202:680–684

    Article  PubMed  Google Scholar 

  81. Farquharson AL, Pranesh N, Witham G et al (2008) A phase II study evaluating the use of concurrent mitomycin C and capecitabine in patients with advanced unresectable pseudomyxome peritonei. Br J Cancer 99:591–596

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  82. Chen CF, Huang CJ, Kang WI et al (2008) Experience with adjuvant chemotherapy for pseudomyxoma peritonei secondary to mucinous adenocarcinoma of the appendix with oxaliplatin/fliotouracil/leucovorin (FOLFOX4). World J Surg Oncol 6:118

    Article  PubMed Central  PubMed  Google Scholar 

  83. Pillai K, Akhter J, Chua TC (2012) Mucolysis by ascorbic acid and hydrogen peroxide on compact mucin secreted in pseudomyxoma peritonei. J Surg Res 174:e69–73

    Article  CAS  PubMed  Google Scholar 

  84. Sun WL, Hutarew G, Gradl J et al (2009) Successfu antiangiogenic combination therapy for pseudomyxoma peritonei with bevacizumab and capecitabine. Cancer Biol Ther 8:1459–1462

    Article  CAS  PubMed  Google Scholar 

  85. Gilbreath JJ, Semino-Mora C, Friedline CJ et al (2013) A core microbiome associated with the peritoneal tumors of pseudomyxoma peritonei. Orphanet J Rare Dis 8:105

    Article  PubMed Central  PubMed  Google Scholar 

  86. Choudry HA, Mavanur A, O’Malley ME et al (2012) Chronic anti-inflammatory drug therapy inhibits gel-forming mucin production in a murine xenograft model of human pseudomyxoma peritonei. Ann Surg Oncol 19:1402–1409

    Article  PubMed Central  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Manuela Robella .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2015 Springer-Verlag Italia

About this chapter

Cite this chapter

Vaira, M., Robella, M., Mellano, A., De Simone, M. (2015). Pseudomyxoma Peritonei. In: Di Giorgio, A., Pinto, E. (eds) Treatment of Peritoneal Surface Malignancies. Updates in Surgery. Springer, Milano. https://doi.org/10.1007/978-88-470-5711-1_16

Download citation

  • DOI: https://doi.org/10.1007/978-88-470-5711-1_16

  • Publisher Name: Springer, Milano

  • Print ISBN: 978-88-470-5710-4

  • Online ISBN: 978-88-470-5711-1

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics