Clinical presentation of the pseudomyxoma peritonei syndrome

Background: Pseudomyxoma peritonei syndrome is characterized by a gradual expansion of mucoid tumour and fluid at specific sites within abdominopelvic regions as a result of a perforated appendiceal adenoma. The aim was to analyse the presenting symptoms and signs of patients with this condition. Methods: Of 410 patients with appendiceal tumours 217 had the diagnosis of pseudomyxoma peritonei syndrome with histological confirmation. A retrospective review of the clinical characteristics that determine presentation was performed. Results: Overall, suspected acute appendicitis was the most common presentation (27 per cent). For women the diagnosis was most commonly made while being evaluated for an ovarian mass (39 per cent). Increasing abdominal girth was the second most common presentation overall (23 per cent). Thirty patients (14 per cent) presented with new-onset hernia, of which the majority (26) were inguinal hernias. Conclusion: Consideration of appendicitis, increased abdominal girth, ovarian mass and new-onset hernia as caused by this syndrome may facilitate diagnosis and definitive treatment.