Urachal carcinoma: A case of a rare neoplasm

Abstract

Introduction: Urachal carcinoma is a rare type of non-urothelial malignancy that arises from the urachal ligament, a remnant of fetal development. It frequently involves the dome of the bladder or its midline, with adenocarcinoma being the most common histological type. This malignancy is generally diagnosed in advanced stages and is associated with poor prognosis. Case report: A 40-year-old woman was referred to hospital due to recurrent urinary tract infections. Imaging studies showed the presence of a 3.7 cm tumor in the bladder dome that extended to the posterior region of the umbilicus. A biopsy through cystoscopy confirmed the diagnosis of urachal carcinoma. Since there were no metastases, the patient underwent partial cystectomy and resection of the urachal ligament and the umbilicus. Surgical margins were negative and it was considered a complete resection. Nine months later, disease progression occurred, with peritoneal carcinomatosis, multiple adenopathies and a 4 cm mass in the pelvic cavity with invasion of the vagina, rectum, and sigmoid colon. She began palliative chemotherapy with cisplatine and 5-fluorouracil. After 7 cycles, progression was again observed, with an increase of the pelvic mass, vaginal and rectal hemorrhage, multiple intramuscular implants, bilateral axillary adenopathies, as well as lesion in the right breast, which was compatible with metastasis from urachal carcinoma. She underwent hemostatic radiotherapy to the pelvic mass and second line palliative chemotherapy with gemcitabine and paclitaxel. After 4 cycles, the patient clinically deteriorated and eventually died. Conclusion: Urachal carcinoma is an aggressive malignancy. Although systemic treatment may be effective in disease control, a standard chemotherapy regimen is yet to be determined. Multicenter trials are needed to clarify the best treatment approach in these patients.