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Difference between revisions of "Urachal carcinoma"
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| 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 | ||
| LMDDx = [[adenocarcinoma of the urinary bladder]], invasive [[urothelial carcinoma]] with glandular differentiation, [[metastatic]] adenocarcinoma | | LMDDx = [[adenocarcinoma of the urinary bladder]], invasive [[urothelial carcinoma]] with glandular differentiation, [[metastatic]] adenocarcinoma (especially colorectal) | ||
| Stains = | | Stains = | ||
| IHC = CK20 +ve, beta-catenin +ve (non-nuclear), p63 -ve, CK34betaE12 +ve | | IHC = CK20 +ve, beta-catenin +ve (non-nuclear), p63 -ve, CK34betaE12 +ve | ||
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| Assdx = | | Assdx = | ||
| Syndromes = | | Syndromes = | ||
| Clinicalhx = | | Clinicalhx = no prior urothelial carcinoma | ||
| Signs = +/-hematuria | | Signs = +/-hematuria | ||
| Symptoms = | | Symptoms = | ||
| Prevalence = very rare | | Prevalence = very rare | ||
| Bloodwork = | | Bloodwork = | ||
| Rads = | | Rads = lesion of bladder dome/anterior bladder, lesion centered in wall of bladder (rather than mucosa) | ||
| Endoscopy = | | Endoscopy = | ||
| Prognosis = usually poor | | Prognosis = usually poor | ||
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}} | }} | ||
'''Urachal carcinoma''' is an uncommon [[cancer|malignant tumour]] that arises from the urachus. Most urachal carcinomas are ''[[adenocarcinoma]]s''. | '''Urachal carcinoma''' is an uncommon [[cancer|malignant tumour]] that arises from the urachus. Most urachal carcinomas are ''[[adenocarcinoma]]s''. | ||
The diagnosis is a clinicopathologic diagnosis - one needs imaging to make it.<ref name=Ref_Amin2-143>{{Ref Amin|2-143}}</ref> | |||
==General== | ==General== | ||
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==Sign out== | ==Sign out== | ||
<pre> | |||
Mass, Urachus, Resection: | |||
- ADENOCARCINOMA, consistent with urachal primary, see comment. | |||
-- Margins clear. | |||
Comment: | |||
The sections show malignant cells within the perivesicular soft tissue arranged in nests and glands with necrosis. The lesion center is within the bladder wall, rather than at the mucosa. The sampled bladder mucosa is within normal limits. | |||
The tumour stains as follows: | |||
POSITIVE: CDX2 (focal, moderate nuclear), CK20 (focal, moderate membranous). | |||
NEGATIVE: CK7, p63, CK5/6, GATA3, beta-catenin (membranous only, nuclei negative). | |||
The histomorphology, immunoprofile and imaging are compatible with a primary urachal adenocarcinoma. | |||
The morphology and immunoprofile of urachal adenocarcinoma overlaps with primary adenocarcinoma of the bladder and somewhat with colorectal adenocarcinoma. Primary adenocarcinoma of the bladder is unlikely as the sampled urothelium is benign, the lesion is centered within the wall and no history of bladder tumours was found. Metastatic colorectal cancer seems unlikely from the clinical information available. | |||
The CAP bladder synoptic is not applicable to urachal carcinoma. | |||
In the context of the "Sheldon Staging System" for urachal carcinoma, this case would be pT3a. | |||
</pre> | |||
===Staging=== | |||
*The CAP urinary bladder synoptic, as per v4.0.2.0, should ''not'' be used. | |||
*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> | *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=== | ====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> | 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. | *pT1 - confined to urachal mucosa. | ||