Hépato-Gastro & Oncologie Digestive
MENUManagement of mucinous appendiceal tumors and pseudomyxoma peritonei Volume 27, issue 2, Février 2020
- Key words: mucinous tumor, pseudomyxoma peritonei, hyperthermia intra-peritoneal chemotherapy (HIPEC), appendix, cytoreductive surgery
- DOI : 10.1684/hpg.2020.1931
- Page(s) : 150-9
- Published in: 2020
Appendiceal mucocele is a progressive distension of the appendix, mainly due to a mucinous tumor. The rupture of the tumor within the abdominal cavity is the origin of pseudomyxoma peritonei (PMP). They are classified into low grade tumors called LAMN (Low grade Appendiceal Mucinous Neoplasms), and high grade tumors called HAMN (High grade Appendiceal Mucinous Neoplasms) and mucinous adenocarcinoma. Appendiceal mucocele is suspected with a symptomatology of appendicitis, whereas patients with PMP often present progressive abdominal distension. The CT scan is the main exam for the diagnosis, although peritoneal MRI is increasingly use to analyze resectability of PMP. In absence of PMP, the management of a mucinous tumor of the appendix is the radical appendicectomy with lymph node dissection. In case of unfavorable histological criteria, a complementary caecectomy or rightcolectomy is necessary. The treatment of PMP combines cytoreductive surgery with hyperthermia intra-peritoneal chemotherapy (HIPEC). Overall survival is 63% at ten years. Main prognostic factors are the histological grade and the completeness of the cytoreductive surgery.