Primary retroperitoneal cystic mucinous carcinoma – case report and review of the literature

Abstract

Primary retroperitoneal mucinous tumours (PRMTs) are rare and predominantly affect females. Due to the rarity of these tumours, histogenesis and management strategy are poorly defined. We report the case of a 29 years old female, who presented with abdominal pain and a palpable mass. Despite complete excision she presented within eight weeks with disseminated disease and a biopsy proven skin metastasis. Despite several courses of chemotherapy she succumbed within 5 months of initial diagnosis. The published literature is reviewed with relevance to the clinical diagnosis, histological assessment and management of this uncommon tumour.