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Case Reports
. 2022 Mar 11;20(1):78.
doi: 10.1186/s12957-022-02548-8.

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

Francesca Ponzini #  1 , Luke Kowal #  1 , Mariam Ghafoor  2 , Allison Goldberg  2 , Joanna Chan  2 , Ryan Lamm  3 , Shawnna M Cannaday  3 , Scott D Richard  4 , Avinoam Nevler  3 , Harish Lavu  3 , Wilbur B Bowne  5 , Norman G Rosenblum  6
Affiliations
Case Reports

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

Francesca Ponzini et al. World J Surg Oncol. .

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.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
A Ultrasound images of the 18-cm mass. B–D Contrast-enhanced perioperative abdominal CT scan of the 18cm mass in the lower abdominal cavity (B axial, C coronal, D sagittal). The imaging revealed a moderately large volume of mucinous ascites (red arrows) and a large right ovarian mass (blue arrows) concerning for cystic teratoma vs malignancy
Fig. 2
Fig. 2
A Mucinous neoplasm, right ovary, showing complex mucinous epithelium (200×). B Hair follicles (red arrow) with adjacent mucinous neoplasm (40×). C SATB2 showing strong nuclear staining (40×). D CDX2/CK20 dual stain showing strong nuclear and cytoplasmic staining, respectively (40×). E CK7 showing focal cytoplasmic staining
See this image and copyright information in PMC

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References

    1. Yan F, Shi F, Li X, Yu C, Lin Y, Li Y, Jin Y. Clinicopathological characteristics of pseudomyxoma peritonei originated from ovaries. Cancer Manage Res. 2020;12:7569–7578. doi: 10.2147/CMAR.S264474. - DOI - PMC - PubMed
    1. Ronnett B, Kurman RJ, Zahn CM, Shmookler BM, Jablonski KA, Kass ME, Sugarbaker PH. Pseudomyxoma peritonei in women: a clinicopathologic analysis of 30 cases with emphasis on site of origin, prognosis, and relationship to ovarian mucinous tumors of low malignant potential. Hum Pathol. 1995;26(5):509–524. doi: 10.1016/0046-8177(95)90247-3. - DOI - PubMed
    1. Hwang JH, So KA, Modi G, Lee JK, Lee NW, Lee KW, Kim I. Borderline-like mucinous tumor arising in mature cystic teratoma of the ovary associated with pseudomyxoma peritonei. Int J Gynecol Pathol. 2009;28:376–380. doi: 10.1097/PGP.0b013e318191e766. - DOI - PubMed
    1. Suh DS, Song YJ, Kwon BS, Lee S, Lee NK, Choi KU, Kim KH. An unusual case of pseudomyxoma peritonei associated with synchronous primary mucinous tumors of the ovary and appendix: a case report. Oncol Lett. 2017;13(6):4813–4817. doi: 10.3892/ol.2017.6079. - DOI - PMC - PubMed
    1. Chicago Consensus Working Group The Chicago Consensus on peritoneal surface malignancies: management of appendiceal neoplasms. Cancer. 2020;126(11):2525–2533. doi: 10.1002/cncr.32881. - DOI - PubMed

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