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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 | ||
Line 13: | Line 13: | ||
| 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> | ||
* | *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. | ||
* | 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: | Image: Urachal carcinoma - very low mag.jpg | UC - very low mag. (WC/Nephron) | ||
Image: | Image: Urachal carcinoma - low mag.jpg | UC - low mag. (WC/Nephron) | ||
Image: | 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== |
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