Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2023 Jan 19:13:1110003.
doi: 10.3389/fonc.2023.1110003. eCollection 2023.

Urachal carcinoma: a large retrospective multicentric study from the French Genito-Urinary Tumor Group

M Guerin  1 , C Miran  2 , E Colomba  3 , M Cabart  4 , T Herrmann  5 , S Pericart  6 , D Maillet  7 , Y Neuzillet  8 , A Deleuze  9 , E Coquan  10 , M Laramas  11 , C Thibault  12 , B Abbar  13 , B Mesnard  14 , D Borchiellini  15 , C Dumont  16 , E Boughalem  17 , J L Deville  18 , M Cancel  19 , C Saldana  20 , A Khalil  21 , G Baciarello  3 , A Flechon  2 , J Walz  22 , G Gravis  1
Affiliations

Urachal carcinoma: a large retrospective multicentric study from the French Genito-Urinary Tumor Group

M Guerin et al. Front Oncol. .

Abstract

Introduction: Urachal cancer (UrC) is a rare, non-urothelial malignancy. Its natural history and management are poorly understood. Although localized to the bladder dome, the most common histological subtype of UrC is adenocarcinoma. UrC develops from an embryonic remnant, and is frequently diagnosed in advanced stage with poor prognosis. The treatment is not standardized, and based only on case reports and small series. This large retrospective multicentric study was conducted by the French Genito-Urinary Tumor Group to gain a better understanding of UrC.

Material and methods: data has been collected retrospectively on 97 patients treated at 22 French Cancer Centers between 1996 and 2020.

Results: The median follow-up was 59 months (range 44-96). The median age at diagnosis was 53 years (range 20-86), 45% were females and 23% had tobacco exposure. For patients with localized disease (Mayo I-II, n=46) and with lymph-node invasion (Mayo III, n=13) median progression-free-survival (mPFS) was 31 months (95% CI: 20-67) and 7 months (95% CI: 6-not reached (NR)), and median overall survival (mOS) was 73 months (95% CI: 57-NR) and 22 months (95% CI: 21-NR) respectively. For 45 patients with Mayo I-III had secondary metastatic progression, and 20 patients were metastatic at diagnosis. Metastatic localization was peritoneal for 54% of patients. Most patients with localized tumor were treated with partial cystectomy, with mPFS of 20 months (95% CI: 14-49), and only 12 patients received adjuvant therapy. Metastatic patients (Mayo IV) had a mOS of 23 months (95% CI: 19-33) and 69% received a platin-fluorouracil combination treatment.

Conclusion: UrC is a rare tumor of the bladder where patients are younger with a higher number of females, and a lower tobacco exposure than in standard urothelial carcinoma. For localized tumor, partial cystectomy is recommended. The mOS and mPFS were low, notably for patients with lymph node invasion. For metastatic patients the prognosis is poor and standard therapy is not well-defined. Further clinical and biological knowledge are needed.

Keywords: multicentric; rare disease; retrospective; urachal cancer; urachus.

PubMed Disclaimer

Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The reviewer JH declared a past co-authorship with the author GG to the handling editor.

Figures

Figure 1
Figure 1
Metastatic sites observed for urachal carcinoma. Chart shows the distribution of metastatic sites among 97 patients with urachal cancer.
Figure 2
Figure 2
Histologic subtypes of urachal cancer. ADK, adenocarcinoma; IHC, immunohistochemistry result.
Figure 3
Figure 3
Overall Survival. The population of 97 patients with urachal cancer at diagnosis is stratified according to the Mayo classification. OS, overall survival; mo, months; NR, Not Reached.
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

References

    1. Hue L, Jacquin M. Cancer colloide de la lombille et de paroi abdominale anterieure ayant envahi la vessie. Union Med la Siene-Inf Rouen (1863) 6:418–20.
    1. Johnson DE, Hodge GB, Abdul-Karim FW, Ayala AG. Urachal carcinoma. Urology (1985) 26(3):218−21. doi: 10.1016/0090-4295(85)90112-8 - DOI - PubMed
    1. Gopalan A, Sharp DS, Fine SW, Tickoo SK, Herr HW, Reuter VE, et al. . Urachal carcinoma: a clinicopathologic analysis of 24 cases with outcome correlation. Am J Surg Pathol (2009) 33(5):659−68. doi: 10.1097/PAS.0b013e31819aa4ae - DOI - PMC - PubMed
    1. El Azzouzi D, Lasseri A. Les Anomalies congénitales de l’ouraque chez l’enfant : le point de vue du chirurgien pédiatre. J Pédiatrie Puéricult (2013) 26(6):301−7. doi: 10.1016/j.jpp.2013.08.004 - DOI
    1. Beck AD, Gaudin HJ, Bonham DG. Carcinoma of the urachus. Br J Urol (1970) 42(5):555−62. doi: 10.1111/j.1464-410X.1970.tb04503.x - DOI - PubMed

LinkOut - more resources