What are the other Names for this Condition? (Also known as/Synonyms)

• Carcinoma of the Urachus

What is Urachal Carcinoma? (Definition/Background Information)

• Urachal Carcinoma is a rare malignancy of the urinary tract that arises from the urachus. According to medical literature, the malignancy constitutes about 0.3% of all bladder cancers and between 10-30% of the bladder adenocarcinomas. Urachal Carcinomas are generally observed in individuals in the 40-70 years’ age group with a slight male predilection being noted
• The urachus is a tubular structure that forms during early fetal development. This structure connects the anterior portion of the urinary bladder to the umbilicus. Usually, the urachus closes by week 12 of gestation, leaving behind a thick fibrous band. However, rarely, some portions of the urachus may not close completely, resulting in a variety of urachal abnormalities. A cancer arising from this region is termed Urachal Carcinoma. The tumor commonly presents mutations in the TP53 gene
• Upon pathological examination, a vast majority of Urachal Carcinomas are non-cystic adenocarcinomas. In some cases, the adenocarcinoma can have cystic features (cystic adenocarcinoma of urachus). Other histologic subtypes have also been reported that include - urothelial carcinoma (UCC) of urachus, squamous cell carcinoma (SCC) of urachus, and neuroendocrine carcinoma (NEC) of urachus
• The tumor is known to grow silently in the region (extraperitoneal location) and may be marked by few initial symptoms. It is very important to establish the urachal origin of the carcinoma, since adenocarcinomas may form in the vicinity of the urachus and invade into it, thereby destroying the urachal remnants. Nevertheless, the overall prognosis of Urachal Carcinoma is generally better than those with conventional bladder adenocarcinomas
• The signs and symptoms may include blood in urine (hematuria), mucus in the urine, burning sensation on urination, abdominal pain, and the presence of a tumor mass. However, in many cases, the condition may be asymptomatic during the early stages. Thus, Urachal Carcinomas are frequently diagnosed after local metastasis has taken place to the pelvic lymph nodes. In some cases, the tumor may metastasize to the bone, lungs, liver, peritoneum, or brain. Also, tumor recurrences have been noted within 2 years of treatment (surgery)
• The mainstay of treatment is a surgical excision of the tumor, which may include complete urachectomy and umbilectomy. In severe cases, partial cystectomy and pelvic lymph node dissection may also be necessary. This may be followed up by chemotherapy and radiation therapy. The prognosis of Urachal Carcinoma is generally poor due to a late diagnosis of the condition; often spread of malignancy to multiple sites are noted and local recurrence risk is very high