Pseudomyxoma peritonei natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Parminder Dhingra, M.D. [2]
Overview
Pseudomyxoma peritonei is a rare disease characterized by the accumulation of mucinous ascites and mucinous tumor disseminated on peritoneal surfaces, mainly abdomen and pelvis; the disease almost always originates from primary appendiceal tumors.
Natural History
If left untreated, patients with pseudomyxoma peritonei may progress to develop abdominal or pelvic pain, bloating, abdominal distension, weight change, and infertility.
- PMP has generally been considered benign; however its behaviour suggests that it should, at best, be considered a borderline malignancy with disease progression over time, to massive abdominal distension and nutritional compromise in most cases. The long term survival in most patients remains poor with reported 5 and 10 year survival rates of 50% and 10%-30%, respectively
Prognosis
- Although pseudomyxoma peritonei as a neoplastic disease runs a chronic, indolent course with late invasion and only rare metastasis outside the peritoneum, it is a serious, recurrent condition with life-threatening complications.[1]
- Biological features of the tumor and access to the current standard of care at specialized oncology centers with a peritoneal surface malignancy program comprise the most important prognostic determinants of pseudomyxoma peritonei.
References
- ↑ Amini, Afshin; Masoumi-Moghaddam, Samar; Ehteda, Anahid; Morris, David (2014). "Secreted mucins in pseudomyxoma peritonei: pathophysiological significance and potential therapeutic prospects". Orphanet Journal of Rare Diseases. 9 (1): 71. doi:10.1186/1750-1172-9-71. ISSN 1750-1172.