Pseudomyxoma peritonei: Diagnostic conundrum
Nandita Kaushal, Mrs. Barkha Sinha
Department of Obstetrics and Gynaecology, West Suffolk NHS foundation Trust
OBJECTIVE:
•Pseudomyxoma peritonei (PMP) is a rare neoplastic condition characterised by gelatinous ascites, which generally arise from mucin-producing appendiceal tumours.
•The diagnostic problem appears when the primary site of origin could be appendix or ovary.
CASE REPORT:
Case 1
•55-year-old lady presented with change in bowel habit and mild bloating in the lower abdomen.
•Blood tests revealed raised CA125 of 43.CT scan confirmed widespread peritoneal disease with mucinous ascites presumably secondary to perforated appendix with large volume disease( mainly pelvis), complex mass and bilateral Kruckenberg’s.
•Underwent complete cytoreduction and HIPEC(Hyperthermic intra-peritoneal chemotherapy). Histology confirmed low-grade mucinous neoplasm and low-grade pseudomyxoma.
•Recurrence within a year after surgery requiring multiple hospitalizations.
Case 2
•52-year-old lady presented with abdominal distension and a palpable mass.
•Blood tests revealed Elevated CA 125( 61), and CEA ( 35). CT scan was conclusive of Left ovarian cystic mass, likely mucinous tumour with distended distal appendix, small amount of pelvic fluids. Despite unilateral involvement of ovaries, there was strong suspicion of PMP.
•Underwent cytoreductive surgery followed by HIPEC. Histology demonstrated low-grade appendiceal mucinous neoplasm with low-grade pseudomyxoma
Remains recurrence-free.
DISCUSSION:
•In most cases of PMP, the appendix is the primary site of the tumour.
•It is crucial to avoid misdiagnosing a secondary ovarian tumour as a primary neoplasm, as this could negatively impact surgical management, additional treatments, and prognosis.1
• PMP is a rare and difficult-to-treat disease, necessitating an optimal management approach that involves a multidisciplinary team, as seen in our case.
CONCLUSIONS:
•The natural course of PMP is characterized by slow but progressive growth, and if untreated, it is ultimately fatal. Despite ongoing challenges, advancements in surgical techniques, perioperative chemotherapy, and emerging therapies provide hope for better outcomes and improved quality of life of PMPpatients.2
REFERENCES:
1.Martins CF, Costa S, Guedes P, Rafael AF. Pseudomyxoma peritonei: a rare tumour mimicking primary ovarian neoplasia. BMJ Case Rep. 2015 Mar 5. doi: 10.1136/bcr-2014-209251
2. Askar A, Arpat A, Durgun V. Pseudomyxoma peritonei: The struggle of a lifetime and the hope of a cure - a rare diagnosis with review of the literature. North Clin Istanb. 2024 Jun 28, Doi: 10.14744/nci.2023.50374.
