Rare occurrence of pseudomyxoma peritonei (PMP) syndrome arising from a malignant transformed ovarian primary mature cystic teratoma treated by cytoreductive surgery and HIPEC: a case report

World J Surg Oncol. 2022 Mar 11;20(1):78. doi: 10.1186/s12957-022-02548-8.

Abstract

Background: Pseudomyxoma peritonei (PMP) syndrome is a disease process that typically occurs from ruptured appendiceal mucocele neoplasms. PMP syndrome arising from malignant transformation of an ovarian primary mature cystic teratoma (MCT) is a pathogenesis rarely encountered.

Case presentation: Herein, we report a 28-year-old patient evaluated and treated for a right ovarian mass and large volume symptomatic abdominopelvic mucinous ascites. Molecular profiling and genetic analysis revealed mutations in ATM, GNAS, and KRAS proteins while IHC demonstrated gastrointestinal-specific staining for CK20, CDX2, CK7, and SATB2. Peritoneal cytology showed paucicellular mucin. Diffuse peritoneal adenomucinosis (DPAM) variant of PMP arising from a ruptured ovarian primary MCT after malignant transformation to a low-grade appendiceal-like mucinous neoplasm was ultimately confirmed. Treatment included staged therapeutic tumor debulking and right salpingo-oophorectomy followed by cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC).

Conclusions: Our report builds upon the existing literature supporting this aggressive treatment option reserved for advanced abdominal malignancies utilized in this patient with a rare clinical entity.

Keywords: Appendiceal mucocele; Cytoreductive surgery (CRS); Disseminated peritoneal adenomucinosis; Hyperthermic intraperitoneal chemotherapy (HIPEC); Ovarian; Pseudomyxoma peritonei; Teratoma.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Cytoreduction Surgical Procedures*
  • Female
  • Humans
  • Hyperthermic Intraperitoneal Chemotherapy*
  • Ovarian Neoplasms* / complications
  • Ovarian Neoplasms* / drug therapy
  • Ovarian Neoplasms* / pathology
  • Ovarian Neoplasms* / surgery
  • Ovariectomy
  • Peritoneal Neoplasms / drug therapy
  • Peritoneal Neoplasms / etiology
  • Peritoneal Neoplasms / pathology
  • Peritoneal Neoplasms / surgery
  • Peritoneum / pathology
  • Peritoneum / surgery
  • Pseudomyxoma Peritonei* / drug therapy
  • Pseudomyxoma Peritonei* / etiology
  • Pseudomyxoma Peritonei* / pathology
  • Pseudomyxoma Peritonei* / surgery
  • Salpingectomy
  • Syndrome
  • Teratoma* / complications
  • Teratoma* / drug therapy
  • Teratoma* / pathology
  • Teratoma* / surgery