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Pseudomyxoma Peritonei
Siva P. Raman, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Pseudomyxoma peritonei (PMP)
          • Definitions

            • Diffuse intraperitoneal accumulation of gelatinous mucinous implants due to rupture of appendiceal mucinous neoplasm
            • Terminology is highly debated and variable, and some authors also use the term PMP for mucinous dissemination after rupture of mucin-producing tumors at other sites (i.e., colon, stomach, fallopian tube, ovary, urachus, etc.)

          IMAGING

          • General Features

            • CT Findings

              • MR Findings

                • Nuclear Medicine Findings

                  • Radiographic Findings

                    • Ultrasonographic Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Staging, Grading, & Classification

                              • Gross Pathologic & Surgical Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        DIAGNOSTIC CHECKLIST

                                        • Consider

                                          Selected References

                                          1. Carr NJ: Current concepts in pseudomyxoma peritonei. Ann Pathol. 34(1):9-13, 2014
                                          2. Diop AD et al: CT imaging of peritoneal carcinomatosis and its mimics. Diagn Interv Imaging. ePub, 2014
                                          3. Vicens RA et al: Multimodality imaging of common and uncommon peritoneal diseases: a review for radiologists. Abdom Imaging. ePub, 2014
                                          4. Buell-Gutbrod R et al: Pathologic diagnosis, origin, and natural history of pseudomyxoma peritonei. Am Soc Clin Oncol Educ Book. 221-5, 2013
                                          5. Faris JE et al: Controversy and consensus on the management of patients with pseudomyxoma peritonei. Curr Treat Options Oncol. 14(3):365-73, 2013
                                          6. Tirumani SH et al: Mucinous neoplasms of the appendix: a current comprehensive clinicopathologic and imaging review. Cancer Imaging. 13:14-25, 2013
                                          7. Krause J et al: Ultrasonography findings and tumour quantification in patients with pseudomyxoma peritonei. Eur J Radiol. 81(4):648-51, 2012
                                          8. Que Y et al: Pseudomyxoma peritonei: some different sonographic findings. Abdom Imaging. 37(5):843-8, 2012
                                          9. Bevan KE et al: Pseudomyxoma peritonei. World J Gastrointest Oncol. 2(1):44-50, 2010
                                          10. Cioppa T et al: Cytoreduction and hyperthermic intraperitoneal chemotherapy in the treatment of peritoneal carcinomatosis from pseudomyxoma peritonei. World J Gastroenterol. 14(44):6817-23, 2008
                                          11. Koyama T et al: Secondary ovarian tumors: spectrum of CT and MR features with pathologic correlation. Abdom Imaging. Epub ahead of print, 2007
                                          12. Jacquemin G et al: Pseudomyxoma peritonei: review on a cluster of peritoneal mucinous diseases. Acta Chir Belg. 105(2):127-33, 2005
                                          13. Takeuchi M et al: Imaging findings of urachal mucinous cystadenocarcinoma associated with pseudomyxoma peritonei. Acta Radiol. 45(3):348-50, 2004
                                          14. Hanbidge AE et al: US of the peritoneum. Radiographics. 23(3):663-84; discussion 684-5, 2003
                                          15. Harshen R et al: Pseudomyxoma peritonei. Clin Oncol (R Coll Radiol). 15(2):73-7, 2003
                                          16. Lo NS et al: Mucinous cystadenocarcinoma of the appendix. The controversy persists: a review. Hepatogastroenterology. 50(50):432-7, 2003
                                          17. Moran BJ et al: The etiology, clinical presentation, and management of pseudomyxoma peritonei. Surg Oncol Clin N Am. 12(3):585-603, 2003
                                          18. Park CM et al: Recurrent ovarian malignancy: patterns and spectrum of imaging findings. Abdom Imaging. 28(3):404-15, 2003
                                          19. Pickhardt PJ et al: Primary neoplasms of the appendix: radiologic spectrum of disease with pathologic correlation. Radiographics. 23(3):645-62, 2003
                                          20. van Ruth S et al: Pseudomyxoma peritonei: a review of 62 cases. Eur J Surg Oncol. 29(8):682-8, 2003
                                          21. Butterworth SA et al: Morbidity and mortality associated with intraperitoneal chemotherapy for Pseudomyxoma peritonei. Am J Surg. 183(5):529-32, 2002
                                          22. Georgescu S et al: Mucinous digestive tumors. Case reports and review of the literature. Rom J Gastroenterol. 11(3):213-8, 2002
                                          23. Nakao A et al: Appendiceal mucocele of mucinous cystadenocarcinoma with a cutaneous fistula. J Int Med Res. 30(4):452-6, 2002
                                          24. Rose MG et al: Typical clinical and radiographic findings in a patient with longstanding malignant pseudomyxoma peritonei secondary to a mucinous adenocarcinoma. Clin Colorectal Cancer. 2(1):59-60, 2002
                                          25. Shappell HW et al: Diagnostic criteria and behavior of ovarian seromucinous (endocervical-type mucinous and mixed cell-type) tumors: atypical proliferative (borderline) tumors, intraepithelial, microinvasive, and invasive carcinomas. Am J Surg Pathol. 26(12):1529-41, 2002
                                          26. Sulkin TV et al: CT in pseudomyxoma peritonei: a review of 17 cases. Clin Radiol. 57(7):608-13, 2002
                                          27. van Ruth S et al: 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. 9(10):961-7, 2002
                                          28. Elias DM et al: Intraperitoneal chemohyperthermia: rationale, technique, indications, and results. Surg Oncol Clin N Am. 10(4):915-33, xi, 2001
                                          29. Sherer DM et al: Pseudomyxoma peritonei: a review of current literature. Gynecol Obstet Invest. 51(2):73-80, 2001
                                          30. Witkamp AJ et al: Rationale and techniques of intra-operative hyperthermic intraperitoneal chemotherapy. Cancer Treat Rev. 27(6):365-74, 2001
                                          31. Yan H et al: Histopathologic analysis in 46 patients with pseudomyxoma peritonei syndrome: failure versus success with a second-look operation. Mod Pathol. 14(3):164-71, 2001
                                          32. Esquivel J et al: Clinical presentation of the Pseudomyxoma peritonei syndrome. Br J Surg. 87(10):1414-8, 2000
                                          33. Nawaz A et al: Pseudomyxoma peritonei manifesting as intestinal obstruction. South Med J. 93(9):891-3, 2000
                                          34. Pestieau SR et al: Pleural extension of mucinous tumor in patients with pseudomyxoma peritonei syndrome. Ann Surg Oncol. 7(3):199-203, 2000
                                          35. Hosch WP et al: Therapy of pseudomyxoma peritonei of appendiceal origin--surgical resection and intraperitoneal chemotherapy. Z Gastroenterol. 37(7):615-22, 1999
                                          36. Wirtzfeld DA et al: Disseminated peritoneal adenomucinosis: a critical review. Ann Surg Oncol. 6(8):797-801, 1999
                                          37. Hinson FL et al: Pseudomyxoma peritonei. Br J Surg. 85(10):1332-9, 1998
                                          Related Anatomy
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                                          Related Differential Diagnoses
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                                          References
                                          Tables

                                          Tables

                                          KEY FACTS

                                          • Terminology

                                            • Imaging

                                              • Pathology

                                                • Clinical Issues

                                                  TERMINOLOGY

                                                  • Abbreviations

                                                    • Pseudomyxoma peritonei (PMP)
                                                  • Definitions

                                                    • Diffuse intraperitoneal accumulation of gelatinous mucinous implants due to rupture of appendiceal mucinous neoplasm
                                                    • Terminology is highly debated and variable, and some authors also use the term PMP for mucinous dissemination after rupture of mucin-producing tumors at other sites (i.e., colon, stomach, fallopian tube, ovary, urachus, etc.)

                                                  IMAGING

                                                  • General Features

                                                    • CT Findings

                                                      • MR Findings

                                                        • Nuclear Medicine Findings

                                                          • Radiographic Findings

                                                            • Ultrasonographic Findings

                                                              • Imaging Recommendations

                                                                DIFFERENTIAL DIAGNOSIS

                                                                  PATHOLOGY

                                                                  • General Features

                                                                    • Staging, Grading, & Classification

                                                                      • Gross Pathologic & Surgical Features

                                                                        CLINICAL ISSUES

                                                                        • Presentation

                                                                          • Demographics

                                                                            • Natural History & Prognosis

                                                                              • Treatment

                                                                                DIAGNOSTIC CHECKLIST

                                                                                • Consider

                                                                                  Selected References

                                                                                  1. Carr NJ: Current concepts in pseudomyxoma peritonei. Ann Pathol. 34(1):9-13, 2014
                                                                                  2. Diop AD et al: CT imaging of peritoneal carcinomatosis and its mimics. Diagn Interv Imaging. ePub, 2014
                                                                                  3. Vicens RA et al: Multimodality imaging of common and uncommon peritoneal diseases: a review for radiologists. Abdom Imaging. ePub, 2014
                                                                                  4. Buell-Gutbrod R et al: Pathologic diagnosis, origin, and natural history of pseudomyxoma peritonei. Am Soc Clin Oncol Educ Book. 221-5, 2013
                                                                                  5. Faris JE et al: Controversy and consensus on the management of patients with pseudomyxoma peritonei. Curr Treat Options Oncol. 14(3):365-73, 2013
                                                                                  6. Tirumani SH et al: Mucinous neoplasms of the appendix: a current comprehensive clinicopathologic and imaging review. Cancer Imaging. 13:14-25, 2013
                                                                                  7. Krause J et al: Ultrasonography findings and tumour quantification in patients with pseudomyxoma peritonei. Eur J Radiol. 81(4):648-51, 2012
                                                                                  8. Que Y et al: Pseudomyxoma peritonei: some different sonographic findings. Abdom Imaging. 37(5):843-8, 2012
                                                                                  9. Bevan KE et al: Pseudomyxoma peritonei. World J Gastrointest Oncol. 2(1):44-50, 2010
                                                                                  10. Cioppa T et al: Cytoreduction and hyperthermic intraperitoneal chemotherapy in the treatment of peritoneal carcinomatosis from pseudomyxoma peritonei. World J Gastroenterol. 14(44):6817-23, 2008
                                                                                  11. Koyama T et al: Secondary ovarian tumors: spectrum of CT and MR features with pathologic correlation. Abdom Imaging. Epub ahead of print, 2007
                                                                                  12. Jacquemin G et al: Pseudomyxoma peritonei: review on a cluster of peritoneal mucinous diseases. Acta Chir Belg. 105(2):127-33, 2005
                                                                                  13. Takeuchi M et al: Imaging findings of urachal mucinous cystadenocarcinoma associated with pseudomyxoma peritonei. Acta Radiol. 45(3):348-50, 2004
                                                                                  14. Hanbidge AE et al: US of the peritoneum. Radiographics. 23(3):663-84; discussion 684-5, 2003
                                                                                  15. Harshen R et al: Pseudomyxoma peritonei. Clin Oncol (R Coll Radiol). 15(2):73-7, 2003
                                                                                  16. Lo NS et al: Mucinous cystadenocarcinoma of the appendix. The controversy persists: a review. Hepatogastroenterology. 50(50):432-7, 2003
                                                                                  17. Moran BJ et al: The etiology, clinical presentation, and management of pseudomyxoma peritonei. Surg Oncol Clin N Am. 12(3):585-603, 2003
                                                                                  18. Park CM et al: Recurrent ovarian malignancy: patterns and spectrum of imaging findings. Abdom Imaging. 28(3):404-15, 2003
                                                                                  19. Pickhardt PJ et al: Primary neoplasms of the appendix: radiologic spectrum of disease with pathologic correlation. Radiographics. 23(3):645-62, 2003
                                                                                  20. van Ruth S et al: Pseudomyxoma peritonei: a review of 62 cases. Eur J Surg Oncol. 29(8):682-8, 2003
                                                                                  21. Butterworth SA et al: Morbidity and mortality associated with intraperitoneal chemotherapy for Pseudomyxoma peritonei. Am J Surg. 183(5):529-32, 2002
                                                                                  22. Georgescu S et al: Mucinous digestive tumors. Case reports and review of the literature. Rom J Gastroenterol. 11(3):213-8, 2002
                                                                                  23. Nakao A et al: Appendiceal mucocele of mucinous cystadenocarcinoma with a cutaneous fistula. J Int Med Res. 30(4):452-6, 2002
                                                                                  24. Rose MG et al: Typical clinical and radiographic findings in a patient with longstanding malignant pseudomyxoma peritonei secondary to a mucinous adenocarcinoma. Clin Colorectal Cancer. 2(1):59-60, 2002
                                                                                  25. Shappell HW et al: Diagnostic criteria and behavior of ovarian seromucinous (endocervical-type mucinous and mixed cell-type) tumors: atypical proliferative (borderline) tumors, intraepithelial, microinvasive, and invasive carcinomas. Am J Surg Pathol. 26(12):1529-41, 2002
                                                                                  26. Sulkin TV et al: CT in pseudomyxoma peritonei: a review of 17 cases. Clin Radiol. 57(7):608-13, 2002
                                                                                  27. van Ruth S et al: 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. 9(10):961-7, 2002
                                                                                  28. Elias DM et al: Intraperitoneal chemohyperthermia: rationale, technique, indications, and results. Surg Oncol Clin N Am. 10(4):915-33, xi, 2001
                                                                                  29. Sherer DM et al: Pseudomyxoma peritonei: a review of current literature. Gynecol Obstet Invest. 51(2):73-80, 2001
                                                                                  30. Witkamp AJ et al: Rationale and techniques of intra-operative hyperthermic intraperitoneal chemotherapy. Cancer Treat Rev. 27(6):365-74, 2001
                                                                                  31. Yan H et al: Histopathologic analysis in 46 patients with pseudomyxoma peritonei syndrome: failure versus success with a second-look operation. Mod Pathol. 14(3):164-71, 2001
                                                                                  32. Esquivel J et al: Clinical presentation of the Pseudomyxoma peritonei syndrome. Br J Surg. 87(10):1414-8, 2000
                                                                                  33. Nawaz A et al: Pseudomyxoma peritonei manifesting as intestinal obstruction. South Med J. 93(9):891-3, 2000
                                                                                  34. Pestieau SR et al: Pleural extension of mucinous tumor in patients with pseudomyxoma peritonei syndrome. Ann Surg Oncol. 7(3):199-203, 2000
                                                                                  35. Hosch WP et al: Therapy of pseudomyxoma peritonei of appendiceal origin--surgical resection and intraperitoneal chemotherapy. Z Gastroenterol. 37(7):615-22, 1999
                                                                                  36. Wirtzfeld DA et al: Disseminated peritoneal adenomucinosis: a critical review. Ann Surg Oncol. 6(8):797-801, 1999
                                                                                  37. Hinson FL et al: Pseudomyxoma peritonei. Br J Surg. 85(10):1332-9, 1998