[Current treatment recommendations for pseudomyxoma peritonei].

BACKGROUND

The term pseudomyxoma peritonei (PMP) describes a clinical syndrome characterized by the presence of gelatinous intraperitoneal accumulation of mucus. It mostly originates from a mucocele of the vermiform appendix. Affected patients are often asymptomatic for a long time. Because of its indolent nature it is usually diagnosed at an advanced stage. Clinical presentation is determined by the dissemination of the tumor.

METHOD

A search and analysis of the current literature were carried out.

RESULTS

Based on the morphological characteristics PMP subtypes with various malignant potential can be differentiated. The prognosis depends on the histopathological differentiation and the clinical stage. The treatment spectrum varies from laparoscopic appendectomy to complete cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC).

CONCLUSION

Due to the rarity of PMP there are no prospective randomized studies. Therefore, there is still controversy regarding the best stage-dependent treatment strategy. This review article attempts to clarify the optimal management of mucinous neoplasms of the appendix and PMP taking the clinical presentation and the histological differentiation into consideration.