Difference between revisions of "Urachal carcinoma"

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| Image      = Urachal_carcinoma_-_high_mag.jpg
| Image      = Urachal_carcinoma_-_high_mag.jpg
| Width      =
| Width      =
| Caption    = Urachal carcinoma. [[H&E stain]].
| Caption    = Urachal carcinoma (right of image) and benign [[urothelium]] (left of image). [[H&E stain]].
| Micro      = atypical cells - usually gland forming, +/-mucinous component, +/-signet rings
| Micro      = atypical cells - usually gland forming, +/-mucinous component, +/-signet rings
| Subtypes  = enteric, mucinous, signet ring
| Subtypes  = enteric, mucinous, signet ring
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| IF        =
| IF        =
| Gross      =
| Gross      =
| Grossing  =
| Grossing  = [[partial cystectomy]]
| Site      = [[urinary bladder]] - specifically the dome
| Site      = urachus, [[urinary bladder]] - specifically the dome
| Assdx      =
| Assdx      =
| Syndromes  =
| Syndromes  =
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==General==
==General==
*Very rare<ref name=pmid16826585>{{Cite journal  | last1 = Ashley | first1 = RA. | last2 = Inman | first2 = BA. | last3 = Sebo | first3 = TJ. | last4 = Leibovich | first4 = BC. | last5 = Blute | first5 = ML. | last6 = Kwon | first6 = ED. | last7 = Zincke | first7 = H. | title = Urachal carcinoma: clinicopathologic features and long-term outcomes of an aggressive malignancy. | journal = Cancer | volume = 107 | issue = 4 | pages = 712-20 | month = Aug | year = 2006 | doi = 10.1002/cncr.22060 | PMID = 16826585 }}</ref>~ 0.2% of bladder cancers.<ref name=pmid22901574>{{Cite journal  | last1 = Bruins | first1 = HM. | last2 = Visser | first2 = O. | last3 = Ploeg | first3 = M. | last4 = Hulsbergen-van de Kaa | first4 = CA. | last5 = Kiemeney | first5 = LA. | last6 = Witjes | first6 = JA. | title = The clinical epidemiology of urachal carcinoma: results of a large, population based study. | journal = J Urol | volume = 188 | issue = 4 | pages = 1102-7 | month = Oct | year = 2012 | doi = 10.1016/j.juro.2012.06.020 | PMID = 22901574 }}</ref>
*Very rare<ref name=pmid16826585>{{Cite journal  | last1 = Ashley | first1 = RA. | last2 = Inman | first2 = BA. | last3 = Sebo | first3 = TJ. | last4 = Leibovich | first4 = BC. | last5 = Blute | first5 = ML. | last6 = Kwon | first6 = ED. | last7 = Zincke | first7 = H. | title = Urachal carcinoma: clinicopathologic features and long-term outcomes of an aggressive malignancy. | journal = Cancer | volume = 107 | issue = 4 | pages = 712-20 | month = Aug | year = 2006 | doi = 10.1002/cncr.22060 | PMID = 16826585 }}</ref>~ 0.2% of bladder cancers.<ref name=pmid22901574>{{Cite journal  | last1 = Bruins | first1 = HM. | last2 = Visser | first2 = O. | last3 = Ploeg | first3 = M. | last4 = Hulsbergen-van de Kaa | first4 = CA. | last5 = Kiemeney | first5 = LA. | last6 = Witjes | first6 = JA. | title = The clinical epidemiology of urachal carcinoma: results of a large, population based study. | journal = J Urol | volume = 188 | issue = 4 | pages = 1102-7 | month = Oct | year = 2012 | doi = 10.1016/j.juro.2012.06.020 | PMID = 22901574 }}</ref>
*Younger <55 years-old.
*Often younger <55 years-old.
*Poor prognosis<ref name=pmid24136277/> - especially for high [[Sheldon stage]].<ref name=pmid19252435>{{Cite journal  | last1 = Gopalan | first1 = A. | last2 = Sharp | first2 = DS. | last3 = Fine | first3 = SW. | last4 = Tickoo | first4 = SK. | last5 = Herr | first5 = HW. | last6 = Reuter | first6 = VE. | last7 = Olgac | first7 = S. | title = Urachal carcinoma: a clinicopathologic analysis of 24 cases with outcome correlation. | journal = Am J Surg Pathol | volume = 33 | issue = 5 | pages = 659-68 | month = May | year = 2009 | doi = 10.1097/PAS.0b013e31819aa4ae | PMID = 19252435 }}</ref>
 
Clinical:
*Hematuria - classic presentation.<ref name=pmid24136277>{{Cite journal  | last1 = Shao | first1 = GJ. | last2 = Cai | first2 = L. | last3 = Li | first3 = XS. | last4 = Song | first4 = G. | last5 = Li | first5 = XY. | last6 = He | first6 = ZS. | last7 = Zhou | first7 = LQ. | title = [Urachal carcinoma: experience of a clinical center within 30 years]. | journal = Beijing Da Xue Xue Bao | volume = 45 | issue = 5 | pages = 774-8 | month = Oct | year = 2013 | doi =  | PMID = 24136277 }}</ref>


Treatment:
Treatment:
*Partial cystectomy +/- umbilectomy.
*[[Partial cystectomy]] +/- umbilectomy.


==Gross==
==Gross==
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==Microscopic==
==Microscopic==
Features:
Features of epithelial malignancy:  
*Atypical cells.
*Atypical (epithelial) cells.
**May be signet ring cells.
**May be [[signet ring cells]].
*Usually gland forming, i.e. adenocarcinoma.
*Usually gland forming, i.e. adenocarcinoma.
*+/-Mucinous component.
*+/-Mucinous component.
*Adjacent urothelium typically benign.
Features making it ''urachal carcinoma'' (modified Sheldon criteria) - all required:<ref name=pmid22301493>{{Cite journal  | last1 = Paner | first1 = GP. | last2 = Barkan | first2 = GA. | last3 = Mehta | first3 = V. | last4 = Sirintrapun | first4 = SJ. | last5 = Tsuzuki | first5 = T. | last6 = Sebo | first6 = TJ. | last7 = Jimenez | first7 = RE. | title = Urachal carcinomas of the nonglandular type: salient features and considerations in pathologic diagnosis. | journal = Am J Surg Pathol | volume = 36 | issue = 3 | pages = 432-42 | month = Mar | year = 2012 | doi = 10.1097/PAS.0b013e31823fe49c | PMID = 22301493 }}</ref>
#Located in bladder dome or anterior wall. †
#Centered on bladder wall (as opposed to the mucosa).
#No widespread cystitis cystica/glandularis outside of the bladder dome and anterior wall.
#No known urothelial carcinoma elsewhere.
 
Notes:
*Gopalan ''et al.''<ref name=pmid19252435/> (based on Sheldon) suggests the following additional/more restrictive criteria:
**Only the dome is allowed. †
**Lesion should be sharply demarcated from the [[urothelium]] of the urinary bladder.
**Urachal remnants should be present in the tumour.


DDx:<ref name=Ref_Amin2-143>{{Ref Amin|2-143}}</ref>
DDx:<ref name=Ref_Amin2-143>{{Ref Amin|2-143}}</ref>
*[[Adenocarcinoma of the urinary bladder]].
*[[Adenocarcinoma of the urinary bladder]].
*Invasive [[urothelial carcinoma]] with glandular differentiation.
*Invasive [[urothelial carcinoma]] with glandular differentiation.
*Metastatic adenocarcinoma/adenocarcinoma extending from another structure, e.g. [[colorectal adenocarcinoma]].
*[[Metastatic]] adenocarcinoma/[[adenocarcinoma]] extending from another structure, e.g. [[colorectal adenocarcinoma]].
*Villous adenoma of the urachus<ref>{{Cite journal  | last1 = Joniau | first1 = S. | last2 = Lerut | first2 = E. | last3 = Van Poppel | first3 = H. | title = A Giant Mucinous Adenocarcinoma Arising within a Villous Adenoma of the Urachus: Case Report and Review of the Literature. | journal = Case Rep Med | volume = 2009 | issue =  | pages = 818646 | month =  | year = 2009 | doi = 10.1155/2009/818646 | PMID = 20182635 }}</ref> - non-invasive.


===Patterns===
===Patterns===
*Enteric - looks like colonic adenocarcinoma.
*Enteric - looks like colonic adenocarcinoma.
*Mucinous.
*Mucinous.
*Signet ring.
*[[signet ring cell carcinoma|Signet ring]].


Note:  
Note:  
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===Images===
===Images===
<gallery>
<gallery>
Image:Urachal_carcinoma_-_very_low_mag.jpg | UC - very low mag. (WC)
Image: Urachal carcinoma - very low mag.jpg | UC - very low mag. (WC/Nephron)
Image:Urachal_carcinoma_-_low_mag.jpg | UC - low mag. (WC)
Image: Urachal carcinoma - low mag.jpg | UC - low mag. (WC/Nephron)
Image:Urachal_carcinoma_-_high_mag.jpg | UC - high mag. (WC)
Image: Urachal carcinoma - intermed mag.jpg | UC - intermed mag. (WC/Nephron)
Image: Urachal carcinoma - high mag.jpg | UC - high mag. (WC/Nephron)
Image: Urachal carcinoma - very high mag.jpg | UC - very high mag. (WC/Nephron)
</gallery>
</gallery>


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Others:<ref name=pmid21572312>{{Cite journal  | last1 = Paner | first1 = GP. | last2 = McKenney | first2 = JK. | last3 = Barkan | first3 = GA. | last4 = Yao | first4 = JL. | last5 = Frankel | first5 = WL. | last6 = Sebo | first6 = TJ. | last7 = Shen | first7 = SS. | last8 = Jimenez | first8 = RE. | title = Immunohistochemical analysis in a morphologic spectrum of urachal epithelial neoplasms: diagnostic implications and pitfalls. | journal = Am J Surg Pathol | volume = 35 | issue = 6 | pages = 787-98 | month = Jun | year = 2011 | doi = 10.1097/PAS.0b013e3182189c11 | PMID = 21572312 }}</ref>
Others:<ref name=pmid21572312>{{Cite journal  | last1 = Paner | first1 = GP. | last2 = McKenney | first2 = JK. | last3 = Barkan | first3 = GA. | last4 = Yao | first4 = JL. | last5 = Frankel | first5 = WL. | last6 = Sebo | first6 = TJ. | last7 = Shen | first7 = SS. | last8 = Jimenez | first8 = RE. | title = Immunohistochemical analysis in a morphologic spectrum of urachal epithelial neoplasms: diagnostic implications and pitfalls. | journal = Am J Surg Pathol | volume = 35 | issue = 6 | pages = 787-98 | month = Jun | year = 2011 | doi = 10.1097/PAS.0b013e3182189c11 | PMID = 21572312 }}</ref>
*p63 -ve (+ve in only 3%).
*[[p63]] -ve (+ve in only 3%).


UC versus [[CRC]] -- not absolute but useful:
UC versus [[CRC]] -- not absolute but useful:
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==Sign out==
==Sign out==
*The diagnosis is a clinicopathologic diagnosis - one needs imaging to make it.<ref name=Ref_Amin2-143>{{Ref Amin|2-143}}</ref>
*The diagnosis is a clinicopathologic diagnosis - one needs imaging to make it.<ref name=Ref_Amin2-143>{{Ref Amin|2-143}}</ref>
*May be [[cancer staging|staged]] with the ''Sheldon system''.<ref name=pmid22901574>{{Cite journal  | last1 = Bruins | first1 = HM. | last2 = Visser | first2 = O. | last3 = Ploeg | first3 = M. | last4 = Hulsbergen-van de Kaa | first4 = CA. | last5 = Kiemeney | first5 = LA. | last6 = Witjes | first6 = JA. | title = The clinical epidemiology of urachal carcinoma: results of a large, population based study. | journal = J Urol | volume = 188 | issue = 4 | pages = 1102-7 | month = Oct | year = 2012 | doi = 10.1016/j.juro.2012.06.020 | PMID = 22901574 }}</ref>
===Sheldon staging system===
Staging as per Sheldon ''et al.'':<ref name=pmid19252435>{{Cite journal  | last1 = Gopalan | first1 = A. | last2 = Sharp | first2 = DS. | last3 = Fine | first3 = SW. | last4 = Tickoo | first4 = SK. | last5 = Herr | first5 = HW. | last6 = Reuter | first6 = VE. | last7 = Olgac | first7 = S. | title = Urachal carcinoma: a clinicopathologic analysis of 24 cases with outcome correlation. | journal = Am J Surg Pathol | volume = 33 | issue = 5 | pages = 659-68 | month = May | year = 2009 | doi = 10.1097/PAS.0b013e31819aa4ae | PMID = 19252435 }}</ref><ref name=pmid6361280>{{Cite journal  | last1 = Sheldon | first1 = CA. | last2 = Clayman | first2 = RV. | last3 = Gonzalez | first3 = R. | last4 = Williams | first4 = RD. | last5 = Fraley | first5 = EE. | title = Malignant urachal lesions. | journal = J Urol | volume = 131 | issue = 1 | pages = 1-8 | month = Jan | year = 1984 | doi =  | PMID = 6361280 }}</ref>
*pT1 - confined to urachal mucosa.
*pT2 - confined to the urachus.
*pT3a - extension into the bladder wall
*pT3b - extension into the abdominal wall.
*pT3c - structures other than the bladder.
*pT4a - metastasis to regional [[lymph node]]s.
*pT4b - metastasis to distant sites.


==See also==
==See also==
[[Urinary bladder]].
*[[Urinary bladder]].
*[[Urothelium]].
*[[Adenocarcinoma of the urinary bladder]].


==References==
==References==
48,790

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