title: [Pseudomyxoma peritonei; a rare tumour that can be treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy].
abstract: Three patients, men aged 47 and 30 and a woman aged 48 years, presented with appendicitis-like symptoms, progressive abdominal distension, and an ovarian pelvic mass, respectively. All three were diagnosed with pseudomyxoma peritonei. Treatment consisted of cytoreductive surgery followed by hyperthermic intraperitoneal chemotherapy (HIPEC). After a mean follow-up of 64 months (range: 19-89) after initial treatment, the second patient had died of disease progression but the other two were alive and free of disease. Pseudomyxoma peritonei is a rare disease, characterised by progressive intraperitoneal accumulation of mucinous ascites, produced by a mucinous tumour mass on the peritoneal surfaces. The primary tumour is usually a mucinous adenoma of the appendix. Treatment by cytoreductive surgery and HIPEC can result in a 5-year survival rate of more than 80%, depending on the histopathology and the completeness of the cytoreduction. Recognising pseudomyxoma peritonei in an early stage, when complete cytoreduction is still achievable, may result in a considerable improvement in survival.
authors.affiliation: Nederlands Kanker Instituut-Antoni van Leeuwenhoek Ziekenhuis, afd. Chirurgie, Amsterdam. r.m.smeenk@amc.uva.nl
authors.forename: R M
authors.initials: RM
authors.lastname: Smeenk
authors.forename: V J
authors.initials: VJ
authors.lastname: Verwaal
authors.forename: F A N
authors.initials: FA
authors.lastname: Zoetmulder
mesh_terms: D000328:Adult; D000970:Antineoplastic Agents / Q000627:therapeutic use; D003131:Combined Modality Therapy; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D006979:Hyperthermia, Induced / Q000379:methods; D008297:Male; D008875:Middle Aged; D010534:Peritoneal Neoplasms / Q000175:diagnosis / Q000188:drug therapy* / Q000601:surgery*; D011553:Pseudomyxoma Peritonei / Q000175:diagnosis / Q000188:drug therapy* / Q000601:surgery*; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents