A Curious Umbilical Fistula: An Unexpected Onset of Urachal Mucinous Cystic Tumour

Abstract

Background: The urachus is an embryonic remnant, which is obliterated in most of the adults. Residual of it persists in 32% of adults and neoplasms, which can arise from this structure, are extremely rare. These are usually diagnosed through incidental findings or for urinary symptoms. Their natural history is characterized by an early metastatization through the peritoneal cavity. Due to their rarity, there are no unanimous consensus for their management and therapy. Radical excision, associated or not with intraperitoneal hypertermic chemotherapy linked to cytoreductive surgery, was suggested over time.Case report: A black woman came to our attention for a gelatinous secretion from the umbilicus. After a MRIstudy, a parietal neoformation of the urachal remnants was diagnosed. This neoformation revealed a cutaneous fistulization and it was excised surgically. Its pathologic exam described the presence of a cystic mucinous urachal tumor. During the follow-up, a cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) was performed, due to the occurence of free intraperitoneal liquid and multiple nodules on the abdominal parenchimas, After the last surgery, the patient underwent radiological and serological follow up and, after 6 months, there are no evidence of pathological recurrence.Discussion and conclusion: In the diagnostic pathway, the MRI has a pivotal role for the study of the peritoneal cavity. Surgically, the complete excision is preferred. The role of the conventional chemotherapy is still under debate, however CRS in association with HIPEC, is certainly useful.