774 - Pseudomyxoma Peritonei: Pathological and Therapeutic aspects of 26 Cases

Abstract

ABSTRACT Introduction Pseudomyxoma peritonei is defined by mucus collection in the peritoneal cavity with or without presence of neoplastic cells. Recent immunohistochemical and cytogenetic applications have confirmed the appendiceal origin. Surgical removal combined with hyperthermic intraperitoneal chemotherapy (HIPEC) is the main treatment. The aim of this study is to evaluate pathological and therapeutic aspects of a Tunisian series of 25 cases. Methods We retrospectively studied 25 patients, in a 17-year period from 1994 to 2011. Data were collected from surgical records and included: Patient presentation, radiologic histological findings, surgical procedure, adjuvant therapy and follow up. Initial and subsequent surgical treatment were conducted at the Salah Azaiez institute. Results The mean age was 38 years (range 32-83 years) and 92% (23/25) were females. The most frequent symptom was abdominal distension (68%). Pathologic specimen showed peritoneal mucinosis carcinomatosis in 40% of patients and dessiminated peritoneal adenomucinosis in 20% of patients. The appendiceal origin was found in 60% of cases and ovarian origin in 28% of cases. All patients have been operated, one patient had neoadjuvant chemotherapy. Only two patients had HIPEC. 52% (13 cases) has post-operative systemic chemotherapy with complete response in 8 cases. Disease recurrence was noted in 48% of patients (12 cases). Second-look surgery has been performed in 8 cases. 8 patients had chemotherapy after recurrence. The median patient follow-up was 36 months (range, 1 to 120 months). 1 and 3-year progression free survival was 40% and 12% respectively. Overall survival at 1, 3, 5 year was 80%, 40%, 20% respectively. One patient developed bone metastases after 13 months of follow up. Conclusion Pseudomyxoma peritonei is a rare disease that must be treated in specialized centers with complete surgical removal, per or immediate post operative intraperitoneal chemotherapy and accurate histological classification. Disclosure All authors have declared no conflicts of interest.