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.
- Wright JL, Porter MP, Li CI, Lange PH, Lin DW: Differences in survival among patients with urachal and nonurachal adenocarcinomas of the bladder. Cancer. 2006, 107: 721-728. 10.1002/cncr.22059.View ArticlePubMedGoogle Scholar
- Sheldon CA, Clayman RV, Gonzalez R, Williams RD, Fraley EE: Malignant urachal lesions. J Urol. 1984, 131: 1-8.PubMedGoogle Scholar
- Siefker-Radtke A: Urachal carcinoma: surgical and chemotherapeutic options. Expert Rev Anticancer Ther. 2006, 6: 1715-1721. 10.1586/1473722.214.171.1245.View ArticlePubMedGoogle Scholar
- Ashley RA, Inman BA, Sebo TJ, Leibovich BC, Blute ML, Kwon ED, Zincke H: Urachal carcinoma: clinicopathologic features and long-term outcomes of an aggressive malignancy. Cancer. 2006, 107: 712-720. 10.1002/cncr.22060.View ArticlePubMedGoogle Scholar
- Pinthus JH, Haddad R, Trachtenberg J, Holowaty E, Bowler J, Herzenberg AM, Jewett M, Fleshner NE: Population based survival data on urachal tumors. J Urol. 2006, 175: 2042-2047. 10.1016/S0022-5347(06)00263-1.View ArticlePubMedGoogle Scholar
- Siefker-Radtke AO, Gee J, Shen Y, Wen S, Daliani D, Millikan RE, Pisters LL: Multimodality management of urachal carcinoma: the MD Anderson Cancer Center experience. J Urol. 2003, 169: 1295-1298. 10.1097/01.ju.0000054646.49381.01.View ArticlePubMedGoogle Scholar
- Herr HW, Bochner BH, Sharp D, Dalbagni G, Reuter VE: Urachal carcinoma: contemporary surgical outcomes. J Urol. 1997, 178: 74-78. 10.1016/j.juro.2007.03.022.View ArticleGoogle Scholar
- Herr HW: Urachal carcinoma: the case for extended partial cystectomy. J Urol. 1994, 151: 365-366.PubMedGoogle Scholar
- Kawakami S, Kageyama Y, Yonese J, Fukui I, Kitahara S, Arai G, Hyouchi N, Suzuki M, Masuda H, Hayashi T, Okuno T, Kihara K: Successful treatment of metastatic adenocarcinoma of the urachus: report of 2 cases with more than 10-year survival. Urology. 2001, 58: 462-10.1016/S0090-4295(01)01259-6.View ArticlePubMedGoogle Scholar
- Logothetis CJ, Samuels ML, Ogden S: Chemotherapy for adenocarcinomas of bladder and urachal origin: 5-fluorouracil, doxorubicin, and mitomycin-C. Urology. 1985, 26: 252-255. 10.1016/0090-4295(85)90121-9.View ArticlePubMedGoogle Scholar
- Ichiyanagi O, Sasagawa I, Suzuki Y, Iijima Y, Kubota Y, Nakada T, Arai S: Successful chemotherapy in a patient with recurrent carcinoma of the urachus. Int Urol Nephrol. 1998, 30: 569-573. 10.1007/BF02550547.View ArticlePubMedGoogle Scholar
- Quilty PM: Urachal carcinoma: a response to chemotherapy. Br J Urol. 1987, 60: 372-10.1111/j.1464-410X.1987.tb04992.x.View ArticlePubMedGoogle Scholar
- Rothenberg ML, et al: Phase I and pharmacokinetic trial of weekly CPT-11. J Clin Oncol. 1993, 11: 2194-2204.PubMedGoogle Scholar
- Hurwitz HI, Fehrenbacher L, Hainsworth JD, Heim W, Berlin J, Holmgren E, Hambleton J, Novotny WF, Kabbinavar F: Bevacizumab in combination with fluorouracil and leucovorin: an active regimen for first-line metastatic colorectal cancer. J Clin Oncol. 2005, 23: 3502-3508. 10.1200/JCO.2005.10.017.View ArticlePubMedGoogle Scholar
- Pozzo C, Barone C, Szanto J, Padi E, Peschel C, Bükki J, Gorbunova V, Valvere V, Zaluski J, Biakhov M, Zuber E, Jacques C, Bugat R: Irinotecan in combination with 5-fluorouracil and folinic acid or with cisplatin in patients with advanced gastric or esophageal-gastric junction adenocarcinoma: results of a randomized phase II study. Ann Oncol. 2004, 15: 1773-1781. 10.1093/annonc/mdh473.View ArticlePubMedGoogle Scholar
- Bouche O, Raoul JL, Bonnetain F, Giovannini M, Etienne PL, Lledo G, Arsène D, Paitel JF, Guérin-Meyer V, Mitry E, Buecher B, Kaminsky MC, Seitz JF, Rougier P, Bedenne L, Milan C, Fédération Francophone de Cancérologie Digestive Group: Randomized multicenter phase II trial of a biweekly regimen of fluorouracil and leucovorin (LV5FU2), LV5FU2 plus cisplatin, or LV5FU2 plus irinotecan in patients with previously untreated metastatic gastric cancer: a Federation Francophone de Cancerologie Digestive Group Study--FFCD 9803. J Clin Oncol. 2004, 22: 4319-4328. 10.1200/JCO.2004.01.140.View ArticlePubMedGoogle Scholar
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.