Intractable Rare Dis Res. 2012;1(3):115-121. (DOI: 10.5582/irdr.2012.v1.3.115)
Pseudomyxoma peritonei as an intractable disease and its preoperative assessment to help improve prognosis after surgery: A review of the literature.
Zhong YS, Deng MH, Xu RY, Kokudo N, Tang W
Pseudomyxoma peritonei (PMP) is a rare and intractable disease with an estimated incidence of one per million population per year. Many aspects of PMP need to be fully and precisely understood; these include its preoperative assessment, i.e. diagnosis, early diagnosis, pathologic classification, and staging according to the peritoneal cancer index, and its surgical treatment. This review focuses on elements of preoperative assessment and surgery using the Sugarbaker procedure to help improve the prognosis for patients with PMP. Accurate data on the incidence of PMP must be based on large populations rather than estimates, and much work needs to be done especially in China. Special attention should be paid to its preoperative assessment. Also proposed here are steps to manage PMP with an emphasis on preoperative assessment.