Pseudomyxoma peritonei

A 55-year-old man presented with a five-month history of increasing abdominal girth, a weight loss of 4.5 kg (10 lb), and occasional episodes of anorexia and nausea. Physical examination revealed possible hepatosplenomegaly as well as a distended abdomen, which was soft, nontender, and dull to percussion. The results of liver-function tests were normal; a serologic test for hepatitis was negative, and the serum level of alpha-fetoprotein was normal. An abdominal plain film, an upper gastrointestinal series, and the results of a barium-enema study were also normal. A computed tomographic (CT) scan of the abdomen, however, revealed multiple, septate, low-attenuation masses throughout the peritoneal space, with scalloping of the hepatic margin (Panel A, arrows), a finding suggestive of pseudomyxoma peritonei. Exploratory laparotomy was performed, followed by right hemicolectomy and excision of large, mesenteric, septate, gelatinous masses -- the so-called jelly belly (Panel B). Pathological examination revealed a ruptured mucinous cystadenoma of the appendix with widespread pseudomyxoma peritonei. After extensive and complete cytoreduction, the patient was given intraperitoneal chemotherapy with mitomycin and fluorouracil. He has been in remission for the past four years and currently shows no clinical signs or CT evidence of a recurrence of the disease.

Barsky S and McGonigle K. N Engl J Med 2004;350:e13