Genomics in pseudomyxoma peritonei

Abstract

Pseudomyxoma peritonei (PMP) syndrome from appendiceal mucinous tumors generically shows a favorable biologic behavior. Cytoreductive surgery plus Hyperthermic IntraPEritoneal Chemotherapy (CRS + HIPEC) is considered the standard treatment for PMP. Despite general good results in terms of overall survival, the disease-free survival and tumor relapse are not uncommon. The relapse is not related only to high-grade PMP histopathology, but often to low-grade PMP diagnosed patients. Histopathology classifications purposed in the last two decades seem not to be the more appropriate tool to predict patients’ outcome. Genomic profiling of PMP is reported by few authors and recently some evidence of a link between Gene Expression Profile (GEP) and outcome prediction of PMP patients has been demonstrated. In this chapter, we review the literature reports and describe the experience of our center about this topic.