Preserving fertility in pseudomyxoma peritonei, a novel approach

Abstract

BACKGROUND: Cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC) is the gold standard treatment for patients with pseudomyxoma peritonei (PMP) but involves routine bilateral salpingo-oophorectomy. Young women wishing to maintain fertility may be reluctant to pursue this. An alternative strategy in women with low-grade PMP has been explored in the form of laparoscopic evacuation of pelvic and ovarian mucin with resection of the appendiceal tumour. METHODS: Between January 2012 and January 2015, four young women (aged 28-35 years) with PMP seeking to maintain fertility underwent laparoscopy, appendicectomy and pelvic mucinous evacuation and washout. Data regarding intra-operative and histopathological findings were collected. Endpoints were fertility-related outcomes and oncological follow-up. RESULTS: Infertility was a presenting symptom in three of the four women. All four had significant pelvic mucinous disease on radiological imaging and were offered CRS and HIPEC as definitive treatment, but chose laparoscopy with appendicectomy and copious irrigation and washout of the pelvis with stripping of mucinous disease off the ovarian surfaces. Postoperative histology demonstrated a low-grade appendiceal mucinous neoplasm (LAMN) in all patients with acellular mucin or low-grade mucinous carcinoma peritonei in the peritoneal cavity. All patients successfully conceived subsequently and gave birth to healthy babies. After 12-29 months follow-up, all women are well with no radiological or laparoscopic evidence of disease recurrence. CONCLUSIONS: In patients with low-grade PMP, initial therapeutic laparoscopy can restore fertility, whilst providing short- to medium-term disease control. This modality in young women wishing to have children appears to be a feasible alternative to immediate CRS and HIPEC.