Treatment of metastatic urachal adenocarcinoma in a young woman: a case report
© Tazi et al; licensee BioMed Central Ltd. 2009
Received: 30 November 2009
Accepted: 4 December 2009
Published: 4 December 2009
A 30-year-old woman with a history of smoking presented with abdominal pain and haematuria. On physical examination, she had a palpable pelvic mass. Imaging revealed a large pelvic mass located on the dome of the bladder, extending from the urachus, with pulmonary metastases. After open biopsy, urachal adenocarcinoma was histologically confirmed. The patient received six cycles of palliative chemotherapy combination 5 fluorouracil and irinotecan with complete response on the pelvic mass and partial response estimated to more than 80% on pulmonary metastasis.
Primary treatment of potentially localized disease includes wide local excision of the urachus, umbilicus, and surrounding soft tissue combined with partial or radical cystectomy and bilateral pelvic lymphadenectomy [4, 5]. Although radical cystectomy has historically been advocated, several studies have demonstrated long-term survival with extended partial cystectomy including en bloc removal of the umbilicus, urachal tumor mass, the entire urachal ligament, and bladder dome [3, 4, 6]. Despite primary treatment, a retrospective analysis of 66 patients with primary urachal carcinoma treated at the Mayo Clinic revealed a 5-year overall survival rate of only 49% [4, 6, 7]. Risk factors for recurrence include lack of en bloc resection of the umbilicus, peritoneal involvement, and positive nodes and/or margins [4, 6, 8]. Owing to lack of early symptoms, the cancer usually presents at an advanced stage. In a population-based study including 62 individuals with a primary diagnosis of urachal tumor, only one patient had cancer localized to the urachus . Rates of local recurrence are high, with disease usually reoccurring in the pelvis or bladder within 2 years of surgery [2, 6]. Chemotherapy and radiation for urachal adenocarcinoma have resulted in minimal responses with no definitive improvement in survival. The rarity of the cancer has prevented accrual to controlled clinical trials. Case reports describe results with various 5-FU and/or cisplatin-based regimens [9–12]. Despite measurable responses to treatment, tumors often recur and the majority of patients die within 2 years of diagnosis. Siefker-Radtke et al. Reported the results of a retrospective review of 42 patients treated at the MD Anderson Cancer Center. Among the 26 patients who developed metastases, only 4 had significant responses to chemotherapy, and of the 9 patients who received chemotherapy with 5-FU or cisplatin-containing regimens three responded . A phase II trial with 5-FU, leucovorin, cisplatin, and gemcitabine for adenocarcinomas of the urachus is ongoing at the MD Anderson Cancer Center . Irinotecan is a topoisomerase I inhibitor that disrupts cell division by interfering with DNA replication. Irinotecan has demonstrated preclinical activity in adenocarcinomas from a variety of tumor types including gastric, colorectal, pancreatic, lung and breast carcinomas . Currently, irinotecan in combination with 5-FU/leucovorin with or without bevacizumab is indicated as first-line therapy for metastatic colorectal cancer . Irinotecan has also demonstrated efficacy for metastatic gastric cancer in combination with 5-FU/leucovorin or cisplatin [15, 16]. Urachal adenocarcinomas are often histologically similar to adenocarcinomas at other sites of origin, including those in then gastrointestinal tract such as the colon or stomach. The histology from this patient demonstrated the typical 'signet-ring' pattern that is also typical of gastric cancer. There is an additional case study in the literature from Japan that describes a patient with metastatic urachal carcinoma and a history of considerable chemotherapy whose lung lesions had a marked response to irinotecan.
Currently, there is no standard chemotherapy regimen for the treatment of metastatic urachal adenocarcinoma. The demonstration that more recently developed agents have efficacy in adenocarcinomas from other tumor types should inspire treatment options for this difficult disease. In addition, agents that demonstrate efficacy in metastatic disease should be pursued in trials designed to clarify the role of neoadjuvant or adjuvant chemotherapy in urachal adenocarcinoma.
Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
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