Preserving fertility in pseudomyxoma peritonei, a novel approach.

Lisa A Sheehan,Sanjeev P Dayal,Faheez Mohamed,Brendan J Moran,Tom D Cecil,Saladin Sawan,Akash M Mehta

doi:10.1515/pp-2016-0024

Abstract

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. 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. 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. 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.

Highlights

Women continue to delay completing their families, likely due to changing socio-economic roles in recent decades, primarily in Western societies
Patients undergoing a therapeutic laparoscopy aimed at assessment and treatment of ovarian and pelvic disease instead of definitive surgical management with Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) were selected for further review
All were found to have pelvic mucinous disease on either radiological imaging or initial laparoscopic evaluation, with limited extra-pelvic disease in two patients. They were counselled about potential risks of disease progression associated with the decision not to undergo CRS and HIPEC

Summary

Introduction

Women continue to delay completing their families, likely due to changing socio-economic roles in recent decades, primarily in Western societies. In 2014 the average age for first time mothers in England and Wales has risen to 28.5 years; for the first time there were more live births to mothers aged 35 or over than to those under 25 years (144,181 vs 138,592 births respectively) [1] This has imposed challenging clinical treatment decisions in young women wishing to maintain fertility and presenting with conditions such as pseudomyxoma peritonei (PMP). Conclusions: In patients with low-grade PMP, initial therapeutic laparoscopy can restore fertility, whilst providing short- to medium-term disease control.

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