ABSTRACT
Background:
Appendix tumors represent about 1% of all gastrointestinal neoplasia, in other words they are quite rare. However, there is a specific type of appendiceal neoplasms (mucinous adenocarcinoma) that spreads to the peritoneum and in almost 20% of the cases, resulting in a disease called pseudomyxoma peritonei. Although, it is a very rare condition, it is nonetheless a very severe one and therefore it is crucial to know how to correctly diagnose and treat it.
Objective:
This study provides updated data on how to diagnose, classify and treat pseudomyxoma peritonei that originates from appendix tumors.
Methods:
A bibliographic research was performed on PubMed database, including articles published since 2000, as well as, cross-referencing with the initial research.
Discussion:
In the past, patients diagnosed with pseudomyxoma peritonei would only undergo palliative measures, so their overall survival rate was greatly reduced. Over the years pseudomyxoma peritonei treatment has evolved and patients are now undergoing treatment which is a combination of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. This new therapy has allowed an increase of survival chances of up to 5 years in those patients with values between 53% and 88%, depending on the type of tumor.
Conclusion:
Despite the great progress we have witnessed in recent years, which have led to a large increase in survival rates, more research needs to be done, on what to do when the disease is in an unresectable stage. Finding a less aggressive therapy than cytoreductive surgery + hyperthermic intraperitoneal chemotherapy will be an important step forward.
Keywords:
Appendix tumors; Pseudomyxoma peritonei; Cancer diagnosis; Cytoreductive surgery; Hyperthermic intraperitoneal chemotherapy