title: New standard of care for appendiceal epithelial neoplasms and pseudomyxoma peritonei syndrome?
abstract: Appendiceal mucinous neoplasms sometimes present with peritoneal dissemination, which was previously a lethal condition with a median survival of about 3 years. Traditionally, surgical treatment consisted of debulking that was repeated until no further benefit could be achieved; systemic chemotherapy was sometimes used as a palliative option. Now, visible disease tends to be removed through visceral resections and peritonectomy. To avoid entrapment of tumour cells at operative sites and to destroy small residual mucinous tumour nodules, cytoreductive surgery is combined with intraperitoneal chemotherapy with mitomycin at 42 degrees C. Fluorouracil is then given postoperatively for 5 days. If the mucinous neoplasm is minimally invasive and cytoreduction complete, these treatments result in a 20-year survival of 70%. In the absence of a phase III study, this new combined treatment should be regarded as the standard of care for epithelial appendiceal neoplasms and pseudomyxoma peritonei syndrome.
authors.affiliation: Washington Cancer Institute, 106 Irving Street, NW Suite 3900 Washington, DC 20020, USA. Paul.Sugarbaker@medstar.net
authors.forename: Paul H
authors.initials: PH
authors.lastname: Sugarbaker
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001063:Appendiceal Neoplasms / Q000188:drug therapy* / Q000473:pathology / Q000601:surgery*; D003131:Combined Modality Therapy; D005472:Fluorouracil / Q000008:administration & dosage; D006801:Humans; D006979:Hyperthermia, Induced; D007263:Infusions, Parenteral; D016685:Mitomycin / Q000008:administration & dosage; D010534:Peritoneal Neoplasms / Q000188:drug therapy* / Q000601:surgery; D011553:Pseudomyxoma Peritonei / Q000188:drug therapy* / Q000601:surgery*; D016019:Survival Analysis; D016896:Treatment Outcome
chemical_list: D016685:Mitomycin; D005472:Fluorouracil