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Images of Memorable Cases: Case 152

Module by: Frederick Moody, Herbert L. Fred, MD, Hendrik A. van Dijk

http://rup.rice.edu/memcases-button.jpg

Case_152-pres1-1.jpg

Case_152-pres1-2.jpg

This 38-year-old, healthy-appearing woman presented with increasing abdominal girth of six months’ duration and a six-day history of a clear-yellow, gooey substance oozing from her umbilicus.

152. Pseudomyxoma peritonei (PMP)

Also known as “jelly belly,” PMP is a unique disorder characterized by the accumulation of gelatinous material throughout the abdomen and pelvis together with mucinous implants on the peritoneal surfaces. It is associated with mucin-producing neoplasms—benign and malignant—originating most often in the appendix and ovary. The chief clinical features are conspicuous gelatinous ascites, no visceral invasion or extraperitoneal spread, indolent course, and striking disparity between the extent of disease and the patient’s general well-being.

Diagnostic confirmation usually requires laparotomy or laparoscopy, because the pathognomonic peritoneal fluid ordinarily is too thick to aspirate through a needle. Treatment consists primarily of repetitive “debulking surgery.” Adjuvant measures include external beam radiotherapy, intraperitoneal radioisotopes, and intraperitoneal or systemic chemotherapy.

The patient shown may be the first with PMP in whom the diagnosis spontaneously “oozed upon the scene.” At laparotomy, a yellowish, mucinous material filled her peritoneal cavity (immediately below) and firmly encased all of her abdominal and pelvic organs (further below). Multiple sections of the peritoneum and umbilicus showed no tumor cells.

Case_152-diag1-1.jpg

Case_152-diag1- 2.jpg

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