Abstract
Pseudomyxoma peritonei (PMP) is a poorly understood disease characterized by mucinous ascites and disseminated peritoneal mucinous tumors, with a clinically protracted course. Although PMP has been ascribed to a variety of sources (Yasar et al. 1997; Lee and Scully 2000; Imaoka et al. 2006), clinical and molecular evidence is mounting that neoplastic mucin-producing goblet cells of the appendix are the primary cause of PMP. (Ronnett et al. 1995; Ronnett et al. 1997; Szych et al. 1999). Although PMP is not an intrinsically malignant process, it is not a benign process either. Not only does PMP replace the entire free space of the abdomen with mucin, it also causes fibrosis that often leads to complete bowel obstruction and ultimately death. Currently the only effective treatment for PMP is cytoreductive surgery (CRS) that removes all of the mucin and mucin-producing cells combined with hyperthermic intraperitoneal chemotherapy (HIPEC) (Sugarbaker 2006).
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Lambert, L.A., Lambert, D.H., Mansfield, P. (2007). Experimental Models and Questions in Basic Science Research for Pseudomyxoma Peritonei. In: González-Moreno, S. (eds) Advances in Peritoneal Surface Oncology. Resent Results in Cancer Research, vol 169. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-30760-0_10
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DOI: https://doi.org/10.1007/978-3-540-30760-0_10
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