Pseudomyxoma peritonei Flashcards
A 53-year-old woman with no previous surgical history undergoes a laparotomy for a 10-cm ovarian mass. Gelatinous material is found on the surface of the right ovary. Pseudomyxoma peritonei is suspected because mucous-like material is found in the cul-de-sac and in the omentum. Frozen section of the ovary is consistent with an atypical proliferating mucinous tumor. The most likely anatomic site of origin of Pseudomyxoma peritonei is:
(A) colon (B) rectum (C) stomach (D) appendix (E) ovary
(D) appendix
What is pseudomyxoma peritonei?
Rare and often fatal - gelatinous ascites and mucinous implants on the peritoneum because the peritoneum transforms into a mucin-secreting epithelium
Usually see in the ovary and appendix but can be associated with colon, rectum, stomach, pancreas, breast, and small intestine tumors too
How does pseudomyxoma peritonei typically present?
Hx/PE: See in females of median age 53 yo; commonly see increase in abdominal girth and pain; abdominal mass or enlarged ovary frequently seen on physical exam
USN: nonmobile echogenic ascites, multiple semi-solid masses
CT: scalloping of organs, multiseptated lesions, omental thickening, mucinous ascites displacing the small intestine
How do tumor markers differ in pseudomyxoma peritonei vs. epithelial ovarian cancer?
PP: significant serum carbohydrate antigen elevation (CA 19-9) and carcinoembryonic antigen (CEA) BUT only mild elevation of CA-125
What are the histological types of pseudomyxoma peritonei?
- disseminated peritoneal adenomucinosis
- peritoneal mucinous carcinomatous
- peritoneal mucinous carcinomatous with intermediate features
How does management differ for pseudomyxoma peritonei vs. other ovarian cancers?
Surgical treatment is aggressive including removal of peritoneum over both hemidiaphragms and pelvic/pericolic peritoneum; also administer IP chemo during and after surgery
Survival is surprisingly good - 86% at 5 years
