[Pseudomyxoma peritonei. A review].
Pseudomyxoma peritonei is a rare disease characterized by the presence of a large mucin component within the abdomen. Recent pathological and genetic advances indicate that they originate from an appendiceal adenoma or adenocarcinoma. Their prognostic is worse than ovarian border-line mucinous tumors (with which they are frequently confused). Currently, the histologic aspect permits to separate the diffuse peritoneal adenomucinosis (DPAM) originating from adenomas, with a relatively benign course, from the mucinous peritoneal carcinomatosis (MPC) originating from adenocarcinomas, with a very poor prognosis. Paradoxically, the treatment of these two diseases are rather similar, with supra-radical surgery as frequently as possible. This type of surgery allows to reach a crude 5-year survival comprised between 50% and 70%, with very different results according to the DPAM-type or the MPC-type. The adjunction of an intraperitoneal chemo-hyperthermia is logical for these two types of disease and probably increases the survival rate.
Elias
D
D
Département de Chirurgie Générale Carcinologique, Institut Gustave Roussy, Villejuif. elias@igr.fr
Sabourin
J C
JC
D000230:Adenocarcinoma / Q000150:complications; D000236:Adenoma / Q000150:complications; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001063:Appendiceal Neoplasms / Q000150:complications; D017024:Chemotherapy, Adjuvant; D003131:Combined Modality Therapy; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008297:Male; D010534:Peritoneal Neoplasms / Q000175:diagnosis / Q000209:etiology / Q000401:mortality / Q000601:surgery; D011379:Prognosis; D011553:Pseudomyxoma Peritonei / Q000175:diagnosis / Q000209:etiology / Q000401:mortality / Q000601:surgery; D018714:Radiotherapy, Adjuvant; D012189:Retrospective Studies; D013997:Time Factors
D000970:Antineoplastic Agents