Pseudomyxoma peritonei of appendiceal origin--a report of seven cases and a review of published reports.

OBJECTIVE

To review our experience of different therapeutic regimens in the treatment of pseudomyxoma peritonei originating in the appendix.

DESIGN

Retrospective study.

SETTING

University hospital, Germany.

SUBJECTS

7 Patients with histologically confirmed pseudomyxoma peritonei.

INTERVENTIONS

Debulking operations.

MAIN OUTCOME MEASURES

Morbidity and mortality.

RESULTS

Complete removal of the tumour was possible at the initial operation only in a patient with a benign tumour. Debulking operations helped the other six patients considerably, but all but one died eventually of complications of the disease (usually bowel obstruction). Two patients developed what were thought to be pulmonary metastases, and one liver metastases, but these were not confirmed histologically. The results of chemotherapy varied; only one patient was free of tumour for about four years and in another progress of the tumour was halted for about a year. Length of follow up ranged from 2-20 years.

CONCLUSIONS

Operation is the treatment of choice for pseudomyxoma peritonei, although complete resection is rarely feasible. Because of the low morbidity and slow growth of the tumour, reoperation is always indicated for symptoms of recurrence.