NIH

Zebra of the Week: Pseudomyxoma peritonei

December 2, 2024

Pseudomyxoma peritonei (PMP) is marked by the progressive buildup of mucin-producing tumors within the peritoneal cavity, leading to symptoms such as increased abdominal girth, pain, and digestive issues. The disease often originates from a ruptured appendiceal tumor, which spreads mucinous cells throughout the abdomen. Diagnosis involves imaging such as CT and MRI, along with histopathological examination to confirm the presence of mucinous tumors. Treatment usually includes cytoreductive surgery to remove visible tumors, followed by hyperthermic intraperitoneal chemotherapy (HIPEC) to target microscopic disease. Despite aggressive treatment, recurrence is common, necessitating regular follow-up and potential repeat interventions. Early detection and specialized care are essential for improving patient outcomes and managing this challenging condition.

Source:

NIH GARD (Genetic and Rare Diseases Information Center). Pseudomyxoma peritonei. Accessed 2024, November 27. https://rarediseases.info.nih.gov/diseases/7488/pseudomyxoma-peritonei