urachal cancer

Urachal cancer usually is an adenocarcinoma (about 90%) mostly with mucinous/colloidal histology. Other rare types include urothelial carcinoma, squamous cell carcinoma, neuroendocrine carcinoma and sarcoma.{{Cite journal|last1=Wright|first1=Jonathan L.|last2=Porter|first2=Michael P.|last3=Li|first3=Christopher I.|last4=Lange|first4=Paul H.|last5=Lin|first5=Daniel W.|date=2006-08-15|title=Differences in survival among patients with urachal and nonurachal adenocarcinomas of the bladder|journal=Cancer|volume=107|issue=4|pages=721–728|doi=10.1002/cncr.22059|issn=0008-543X|pmid=16826584|doi-access=}}{{Cite journal|last1=Reis|first1=Henning|last2=Krafft|first2=Ulrich|last3=Niedworok|first3=Christian|last4=Módos|first4=Orsolya|last5=Herold|first5=Thomas|last6=Behrendt|first6=Mark|last7=Al-Ahmadie|first7=Hikmat|last8=Hadaschik|first8=Boris|last9=Nyirady|first9=Peter|last10=Szarvas|first10=Tibor|date=2018|title=Biomarkers in Urachal Cancer and Adenocarcinomas in the Bladder: A Comprehensive Review Supplemented by Own Data|journal=Disease Markers|volume=2018|pages=7308168|doi=10.1155/2018/7308168|issn=1875-8630|pmc=5867586|pmid=29721106|doi-access=free }}

Urachal cancer can exist for some years without any symptoms. The most frequent initial symptom is haematuria which occurs when the urachal tumour has penetrated the bladder wall, but mucinuria (mucin in the urine), local pain or swelling, recurrent local or urinary tract infections and umbilical discharge can (but is not always) be seen.

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According to the American Urological Association, the diagnostic criteria are the following:{{Cite web|url=https://www.auanet.org/education/auauniversity/education-products-and-resources/pathology-for-urologists/urinary-bladder/non-urothelial-carcinomas/urachal-adenocarcinoma|title=Urachal Adenocarcinoma|website=AUAnet.org}}

1. The location of the tumor is mostly at the bladder dome.
2. No findings of cystitis glandularis on the bladder surface. These findings can be precursor lesions of a primary bladder adenocarcinoma.
3. No history of a different primary adenocarcinoma with the same morphology.

Tissue for histological analysis is usually obtained via a transurethral resection of bladder tumor (TURBT).

File:UrC CDX2 200x.tif-positivity with typical nuclear staining. 200x magnification.|left]]

According to the current version (4th ed.) of the WHO classification of Tumours of the Urinary System, the diagnosis of a urachal adenocarcinoma usually needs a location of the tumor in the bladder dome and/or anterior wall, epicenter of the tumor in the bladder wall, absence of widespread cystitis cystica and/or cystitis glandularis beyond the dome or anterior wall, and absence of another similar structured tumor as a primary tumor elsewhere in the body. A urachal remnant in association with the tumor can help with the diagnosis.{{Cite journal|last1=Humphrey|first1=Peter A.|last2=Moch|first2=Holger|last3=Cubilla|first3=Antonio L.|last4=Ulbright|first4=Thomas M.|last5=Reuter|first5=Victor E.|date=July 2016|title=The 2016 WHO Classification of Tumours of the Urinary System and Male Genital Organs—Part B: Prostate and Bladder Tumours|url=http://dx.doi.org/10.1016/j.eururo.2016.02.028|journal=European Urology|volume=70|issue=1|pages=106–119|doi=10.1016/j.eururo.2016.02.028|pmid=26996659|issn=0302-2838}}{{Cite journal|last1=Gopalan|first1=Anuradha|last2=Sharp|first2=David S.|last3=Fine|first3=Samson W.|last4=Tickoo|first4=Satish K.|last5=Herr|first5=Harry W.|last6=Reuter|first6=Victor E.|last7=Olgac|first7=Semra|date=May 2009|title=Urachal Carcinoma|url= |journal=American Journal of Surgical Pathology|volume=33|issue=5|pages=659–668|doi=10.1097/pas.0b013e31819aa4ae|pmid=19252435|issn=0147-5185|pmc=4225778}}{{Cite journal|last1=Herr|first1=Harry W.|last2=Bochner|first2=Bernard H.|last3=Sharp|first3=David|last4=Dalbagni|first4=Guido|last5=Reuter|first5=Victor E.|date=July 2007|title=Urachal Carcinoma: Contemporary Surgical Outcomes|url=http://dx.doi.org/10.1016/j.juro.2007.03.022|journal=Journal of Urology|volume=178|issue=1|pages=74–78|doi=10.1016/j.juro.2007.03.022|pmid=17499279|issn=0022-5347}}{{Cite journal|last1=Sheldon|first1=Curtis A.|last2=Clayman|first2=Ralph V.|last3=Gonzalez|first3=Ricardo|last4=Williams|first4=Richard D.|last5=Fraley|first5=Elwin E.|date=January 1984|title=Malignant Urachal Lesions|url=http://dx.doi.org/10.1016/s0022-5347(17)50167-6|journal=Journal of Urology|volume=131|issue=1|pages=1–8|doi=10.1016/s0022-5347(17)50167-6|pmid=6361280|issn=0022-5347}} In addition, other diagnostic systems also for urachal carcinomas that are not adenocarcinomas have been proposed.{{Cite journal|last1=Paner|first1=Gladell P.|last2=Barkan|first2=Güliz A.|last3=Mehta|first3=Vikas|last4=Sirintrapun|first4=Sahussapont Joseph|last5=Tsuzuki|first5=Toyonori|last6=Sebo|first6=Thomas J.|last7=Jimenez|first7=Rafael E.|date=March 2012|title=Urachal carcinomas of the nonglandular type: salient features and considerations in pathologic diagnosis|url=https://pubmed.ncbi.nlm.nih.gov/22301493|journal=The American Journal of Surgical Pathology|volume=36|issue=3|pages=432–442|doi=10.1097/PAS.0b013e31823fe49c|issn=1532-0979|pmid=22301493|s2cid=5277019}}

Immunohistochemical stains are usually less helpful in the histopathological differential diagnosis of urachal adenocarcinomas.{{Cite journal|last1=Reis|first1=H.|last2=Szarvas|first2=T.|date=June 2019|title=Urachal cancer-current concepts of a rare cancer|url=https://pubmed.ncbi.nlm.nih.gov/30895340|journal=Der Pathologe|volume=40|issue=Suppl 1|pages=31–39|doi=10.1007/s00292-018-0516-9|issn=1432-1963|pmid=30895340|s2cid=84185828}}

CT and MRI scans are useful to evaluate local invasion and metastasis to lymph nodes and other parts of the body. Besides, in 32 to 46% of the cases, they show calcifications which is very suggestive of the disease.{{Cite journal |last1=Claps |first1=Mélanie |last2=Stellato |first2=Marco |last3=Zattarin |first3=Emma |last4=Mennitto |first4=Alessia |last5=Sepe |first5=Pierangela |last6=Guadalupi |first6=Valentina |last7=Mennitto |first7=Roberta |last8=de Braud |first8=Filippo G.M. |last9=Verzoni |first9=Elena |last10=Procopio |first10=Giuseppe |date=January 2020 |title=Current Understanding of Urachal Adenocarcinoma and Management Strategy |url=http://link.springer.com/10.1007/s11912-020-0878-z |journal=Current Oncology Reports |language=en |volume=22 |issue=1 |pages=9 |doi=10.1007/s11912-020-0878-z |pmid=31989430 |s2cid=210925794 |issn=1523-3790}}

Surgical management is en bloc resection of bladder, urachal remnant, and umbilicus. In progressed stages, radiotherapy seems not to lead to sufficient response rates. However, chemotherapy regimes containing 5-FU (and Cisplatin) have been described to be useful in these cases.{{Cite journal|last=Siefker-Radtke|first=Arlene|date=2012-10-01|title=Urachal adenocarcinoma: a clinician's guide for treatment|journal=Seminars in Oncology|volume=39|issue=5|pages=619–624|doi=10.1053/j.seminoncol.2012.08.011|issn=1532-8708|pmid=23040259}} In recent years, targeted therapies have been demonstrated to be useful in reports of single cases. These agents included Sunitinib,{{Cite journal|last1=Testa|first1=Isabella|last2=Verzoni|first2=Elena|last3=Grassi|first3=Paolo|last4=Colecchia|first4=Maurizio|last5=Panzone|first5=Filomena|last6=Procopio|first6=Giuseppe|date=2014-10-27|title=Response to targeted therapy in urachal adenocarcinoma|journal=Rare Tumors|volume=6|issue=4|pages=5529|doi=10.4081/rt.2014.5529|issn=2036-3605|pmc=4274441|pmid=25568747}} Gefitinib,{{Cite journal|last1=Goss|first1=G.|last2=Hirte|first2=H.|last3=Miller|first3=W. H.|last4=Lorimer|first4=I. a. J.|last5=Stewart|first5=D.|last6=Batist|first6=G.|last7=Parolin|first7=D. a. E.|last8=Hanna|first8=P.|last9=Stafford|first9=S.|date=2005-03-01|title=A phase I study of oral ZD 1839 given daily in patients with solid tumors: IND.122, a study of the Investigational New Drug Program of the National Cancer Institute of Canada Clinical Trials Group|journal=Investigational New Drugs|volume=23|issue=2|pages=147–155|doi=10.1007/s10637-005-5860-y|issn=0167-6997|pmid=15744591|s2cid=20979000}} Bevacizumab{{Cite journal|last1=Kanamaru|first1=Tomohiro|last2=Iguchi|first2=Taro|last3=Yukimatsu|first3=Nao|last4=Shimizu|first4=Yasuomi|last5=Kohyama|first5=Yuki|last6=Tachibana|first6=Hirokazu|last7=Kato|first7=Minoru|last8=Yamasaki|first8=Takeshi|last9=Tamada|first9=Satoshi|date=2015-03-01|title=A Case of Metastatic Urachal Carcinoma Treated With FOLFIRI (irinotecan and 5-Fluorouracil/leucovorin) Plus Bevacizumab|journal=Urology Case Reports|volume=3|issue=2|pages=9–11|doi=10.1016/j.eucr.2014.11.004|issn=2214-4420|pmc=4714276|pmid=26793485}} and Cetuximab.{{Cite journal|last1=Collazo-Lorduy|first1=Ana|last2=Castillo-Martin|first2=Mireia|last3=Wang|first3=Li|last4=Patel|first4=Vaibhav|last5=Iyer|first5=Gopa|last6=Jordan|first6=Emmet|last7=Al-Ahmadie|first7=Hikmat|last8=Leonard|first8=Issa|last9=Oh|first9=William K.|date=2016-05-10|title=Urachal Carcinoma Shares Genomic Alterations with Colorectal Carcinoma and May Respond to Epidermal Growth Factor Inhibition|journal=European Urology|volume=70|issue=5|pages=771–775|doi=10.1016/j.eururo.2016.04.037|issn=1873-7560|pmc=5489411|pmid=27178450}}

Measurement of serum concentrations of CEA, CA19-9 and CA125 can be helpful in monitoring urachal cancer{{Cite journal|last1=Siefker-Radtke|first1=Arlene O.|last2=Gee|first2=Jason|last3=Shen|first3=Yu|last4=Wen|first4=Sijin|last5=Daliani|first5=Danai|last6=Millikan|first6=Randall E.|last7=Pisters|first7=Louis L.|date=2003-04-01|title=Multimodality management of urachal carcinoma: the M. D. Anderson Cancer Center experience|journal=The Journal of Urology|volume=169|issue=4|pages=1295–1298|doi=10.1097/01.ju.0000054646.49381.01|issn=0022-5347|pmid=12629346}}

The 5-year survival is estimated between 25 and 61%. Worse prognostic factors include the presence of residual tumor at the margin of the resection specimen (R+), invasion of the peritoneum and metastatic disease.

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• [http://rarediseases.info.nih.gov/GARD/Disease.aspx?PageID=4&diseaseID=7836 Urachal cancer] page from the National Institutes of Health
• [https://patients.uroweb.org/urachal-cancer/ Urachal cancer] page from the [https://patients.uroweb.org/ European Association of Urology - Patient Information]
• [https://urachalcancer.org/ Urachal cancer] page from urachal cancer researchers ([https://urachalcancer.org/ UrachalCancer.org])

{{Urologic neoplasia}}

Category:Urological neoplasia