Prepared by Herbert L. Fred, MD, Professor, and Charles Talakkottur, MD, Resident, Department of Internal Medicine, The University of Texas Health Science Center at Houston
A 38-year-old woman presented with a 6-day history of a painless umbilical discharge. On physical examination, she looked well but had conspicuous ascites and a jellylike substance oozing from an ulcerated umbilicus. At laparotomy, the same type of substance filled her peritoneal cavity (Figure) and bound together all of her abdominal and pelvic organs. These findings were diagnostic of pseudomyxoma peritonei. No tumor cells were evident in sections of the peritoneum or the umbilicus.
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| Figure—Jellylike substance filling the peritoneal cavity. |
Points to remember: pseudomyxoma peritonei—also known as "jelly belly"—is a rare disorder characterized by diffuse collections of gelatinous material in the abdomen and pelvis along with mucinous implants on the peritoneal surfaces. It is associated with mucin-producing neoplasms—benign and malignant—originating most often in the appendix or in the ovary. The chief clinical features are gelatinous ascites, indolent course, and striking disparity between the extent of disease and the patient's healthy appearance. Repetitive "debulking surgery" is the mainstay of treatment; the 5-year survival rate is about 40%.
Diagnosis: Pseudomyxoma peritonei.