Rare Case Report of Primary Pseudomyxoma Ovarii with Disseminated Peritoneal Adenomucinosis

  • Renuka GuptaEmail author
  • Shruti Bhatia
  • Amit Gupta
  • Sheena Khetarpal Bajaj
Case Report


Pseudomyxoma peritonei (PMP) is a poorly understood uncommon condition with an estimated incidence of one to two per million (worldwide) per year. It is characterized by the peritoneal deposition of mucinous implants, diffusely involving the peritoneal surfaces, most commonly of the appendix, and occasionally from the ovary, coupled with mucinous ascites. PMP associated with primary ovarian mucinous tumor was very rare. This is a case report of a 45-year-old female presented with large ovarian mass with gross ascites which was operated and diagnosed as primary atypical proliferative mucinous tumor with pseudomyxoma ovarii with disseminated peritoneal adenomucinosis.


Pseudomyxoma peritonei (PMP) Atypical proliferative mucinous tumor Pseudomyxoma ovarii Disseminated peritoneal adenomucinosis (DPAM) 


Compliance with Ethical Standards

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical Approval

This article does not contain any studies with human participants or animals performed by any of the authors as this is a retrospective case report only.

Informed Consent

Informed consent from patient was taken.


  1. 1.
    Moran BJ, Cecil TD. The etiology, clinical presentation, and management of pseudomyxoma peritonei. Surg Oncol Clin N Am. 2003;12:585–603.CrossRefPubMedGoogle Scholar
  2. 2.
    Smeenk RM, van Velthuysen ML, Verwaal VJ, Zoetmulder FA. Appendiceal neoplasms and pseudomyxoma peritonei: a population based study. Eur J Surg Oncol. 2008;34:196–201.CrossRefPubMedGoogle Scholar
  3. 3.
    Al-Buainain H, Al-Jehani Y, Moghazy K, Al-Quorain A. Psuedomyxoma peritonei secondary to adenocarcinoma of the cecum. J Fam Community Med. 2010;17(2):103–6.CrossRefGoogle Scholar
  4. 4.
    Werth R. Klinische und anatomische Untersuchungen zur Lehre von den Bauchgeschwullsten und der Laparotomie [Clinical and anatomical studies in teaching abdominal tumors and laparotomy]. Arch Gynecol Obstet. 1884;24:100–18.Google Scholar
  5. 5.
    Franckel E. Uber das sogennate pseudomyxome peritonei [About the so called pseudomyxoma peritonei]. Med Wochenschr. 1901;48:965–70.Google Scholar
  6. 6.
    O’Connell JT, Tomlinson JS, Roberts AA, McGonigle KF, Barsky SH. Pseudomyxoma peritonei is a disease of MUC2-expressing goblet cells. Am J Pathol. 2002;161(2):551–64.CrossRefPubMedPubMedCentralGoogle Scholar
  7. 7.
    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;19:1390–408.CrossRefPubMedGoogle Scholar
  8. 8.
    Li C, Kanthan R, Kanthan SC. Pseudomyxoma peritonei—a revisit: report of 2 cases and literature review. World J Surg Oncol. 2006;4:60.CrossRefPubMedPubMedCentralGoogle Scholar
  9. 9.
    Järvinen P, Lepistö A. Clinical presentation of pseudomyxoma peritonei. Scand J Surg. 2010;99(4):213–6.CrossRefPubMedGoogle Scholar
  10. 10.
    Zissin R, Gayer G, Fishman A, Edelstein E, Shapiro-Feinberg M. Synchronous mucinous tumors of the ovary and the appendix associated with pseudomyxoma peritonei: CT findings. Abdom Imaging. 2000;25:311–6.CrossRefPubMedGoogle Scholar
  11. 11.
    Baratti D, Kusamura S, Nonaka D, Cabras AD, Laterza B, Deraco M. Pseudomyxoma peritonei: biological features are the dominant prognostic determinants after complete cytoreduction and hyperthermic intraperitoneal chemotherapy. Ann Surg. 2009;249:243–9.CrossRefPubMedGoogle Scholar
  12. 12.
    Chuaqui RF, Zhuang Z, Emmert-Buck MR, Bryant BR, Nogales F, Tavassoli FA, Merino MJ. Genetic analysis of synchronous mucinous tumors of the ovary and appendix. Hum Pathol. 1996;27:165–71.CrossRefPubMedGoogle Scholar
  13. 13.
    Rouzbahman M, Chetty R. Mucinous tumours of appendix and ovary: an overview and evaluation of current practice. J Clin Pathol. 2014;67:193–7.CrossRefPubMedGoogle Scholar
  14. 14.
    Hart WR. Mucinous tumors of the ovary: a review. Int J Gynecol Pathol. 2005;24:4–25.PubMedGoogle Scholar
  15. 15.
    Yemelyanova AV, Vang R, Judson K, Wu LS, Ronnett BM. Distinction of primary and metastatic mucinous tumors involving the ovary: analysis of size and laterality data by primary site with reevaluation of an algorithm for tumor classification. Am J Surg Pathol. 2008;32:128–38.CrossRefPubMedGoogle Scholar
  16. 16.
    Zaino RJ, Brady MF, Lele SM, Michael H, Greer B, Bookman MA. Advanced stage mucinous adenocarcinoma of the ovary is both rare and highly lethal: a gynecologic oncology group study. Cancer. 2011;117:554–62.CrossRefPubMedGoogle Scholar
  17. 17.
    Nonaka D, Kusamura S, Baratti D, Casali P, Younan R, Deraco M. CDX-2 expression in pseudomyxoma peritonei: a clinicopathological study of 42 cases. Histopathology. 2006;49:381–7.CrossRefPubMedGoogle Scholar
  18. 18.
    Zhou F, Chen X, Li Y, Huang L. Two independent primary mucinous tumors involving the appendix and ovary accompanied with acellular pseudomyxoma peritonei. Int J Clin Exp Pathol. 2015;8:11831–4.PubMedPubMedCentralGoogle Scholar
  19. 19.
    Levy AD, Shaw JC, Sobin LH. Secondary tumors and tumorlike lesions of the peritoneal cavity: imaging features with pathologic correlation. Radiographics. 2009;29:347–73.CrossRefPubMedGoogle Scholar
  20. 20.
    Baratti D, Kusamura S, Milione M, Pietrantonio F, Caporale M, Guaglio M, Deraco M. Pseudomyxoma peritonei of extra-appendiceal origin: a comparative study. Ann Surg Oncol. 2016;23:4222–30.CrossRefPubMedGoogle Scholar

Copyright information

© Association of Gynecologic Oncologists of India 2018

Authors and Affiliations

  1. 1.Department of Gynae-OncologyAction Cancer HospitalNew DelhiIndia
  2. 2.Department of RadiologyPentamed HospitalNew DelhiIndia
  3. 3.Action Cancer HospitalNew DelhiIndia

Personalised recommendations