PSEUDOMYXOMA PERITONEI ASSOCIATED WITH OVARIAN MUCINOUS CYSTADENOMA: DIAGNOSTIC CHALLENGES IN DETERMINING THE PRIMARY ORIGIN

Thị Yến Lê1, , Văn Hồng Nguyễn1, Thị Thảo Phương Phạm1, Tuấn Dũng Trịnh1
1 Bệnh viện Đa khoa Tâm Anh

Main Article Content

Abstract

Background: Pseudomyxoma peritonei (PMP) is a clinical term referring to diffuse intraperitoneal accumulation of mucin secondary to mucinous neoplasms. It remains a controversial entity with respect to biological behavior and nomenclature. The majority of cases originate from appendiceal mucinous neoplasms. However, when PMP is associated with a morphologically benign ovarian mucinous tumor, determining the primary site becomes a significant diagnostic challenge.


Case presentation: A 32-year-old woman was incidentally found to have a left ovarian mass during a routine prenatal examination. Ultrasound and MRI revealed a large multilocular cystic lesion associated with free intraperitoneal fluid (ORADS 4–5). The patient underwent left oophorectomy, partial right oophorectomy, and appendectomy. Grossly, the tumor was a cystic mass filled with mucinous material. Histologically, it was consistent with mucinous cystadenoma lined by benign-appearing mucinous columnar epithelium. Peritoneal nodules, omentum, and small bowel specimens demonstrated abundant low-cellularity mucin without cytologic atypia. No neoplasm was identified in the appendix.


Conclusion: The final diagnosis was left ovarian mucinous cystadenoma associated with grade 1 pseudomyxoma peritonei. This case raises the question of whether the intraperitoneal mucin originated from a benign ovarian tumor or from an undetected primary lesion elsewhere. Comprehensive histopathological evaluation and a systematic diagnostic approach are essential for determining the origin of the disease.

Article Details

References

1. Moran BJ, Cecil TD. The etiology, clinical presentation, and management of pseudomyxoma peritonei. Surg Oncol Clin N Am. 2003 Jul;12(3):585-603. 
2. Mittal R, Chandramohan A, Moran B. Pseudomyxoma peritonei: natural history and treatment. Int J Hyperthermia. 2017 Aug;33(5):511-519. 
3. Murage NW, Ahmed NM, Underwood TJ, et al. The genetic profile and molecular subtypes of human pseudomyxoma peritonei and appendiceal mucinous neoplasms: a systematic review. Cancer Cell International. 2023;42:335–359.
4. Norman J. Carr, Cecil TD, Mohamed F, et al.
A consensus for classification and athologic reporting of pseudomyxoma peritonei and associated appendiceal neoplasia: The results of the Peritoneal Surface Oncology Group International (PSOGI) modified Delphi process. American Journal of Surgical Pathology. 2016;40(1):14–26.
5. WHO Classification of Tumours Editorial Board. Digestive System Tumours. 5th ed. Lyon (France): International Agency for Research on Cancer; 2019.
6. Govaerts K, Chandrakumaran K, Carr NJ, et al. Appendiceal tumours and pseudomyxoma peritonei: literature review with PSOGI/EURACAN clinical practice guidelines for diagnosis and treatment. European Journal of Surgical Oncology. 2021;47(1):11-35. doi:10.1016/j.ejso.2020.02.012.