Pseudomyxoma peritonei most commonly arises from which primary site and what is its characteristic macroscopic finding?

• A High-grade serous ovarian carcinoma with mucin-producing metastases
• B Ruptured mucinous ovarian cystadenoma depositing epithelial cells throughout peritoneum
• C Colonic mucinous adenocarcinoma with peritoneal spread
• D Mucinous cystadenoma or low-grade appendiceal mucinous neoplasm (LAMN) spreading to peritoneum as jelly-belly appearance ✓

Explanation

Pseudomyxoma peritonei (PMP) is most commonly (>90%) caused by low-grade appendiceal mucinous neoplasm (LAMN), which ruptures or perforates and seeds the peritoneum with mucin-secreting epithelial cells, creating a 'jelly-belly' abdomen filled with gelatinous mucin. Although ovarian mucinous tumors are often involved, modern molecular studies confirm the appendix as the primary site in most cases. Management is by cytoreductive surgery + hyperthermic intraperitoneal chemotherapy (HIPEC) in specialized centers. The Ronnett classification divides PMP into disseminated peritoneal adenomucinosis (DPAM) and peritoneal mucinous carcinomatosis (PMCA).

Reference: Shaw's Textbook of Gynaecology, 17th ed.

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Written and medically reviewed by the StethoPrep medical team.