Difference between revisions of "Urachal carcinoma"

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*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.
*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 presenation.<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:

Revision as of 04:32, 24 November 2014

Urachal carcinoma
Diagnosis in short

Urachal carcinoma (right of image) and benign urothelium (left of image). H&E stain.
LM atypical cells - usually gland forming, +/-mucinous component, +/-signet rings
Subtypes enteric, mucinous, signet ring
LM DDx adenocarcinoma of the urinary bladder, invasive urothelial carcinoma with glandular differentiation, metastatic adenocarcinoma
IHC CK20 +ve, beta-catenin +ve (non-nuclear), p63 -ve, CK34betaE12 +ve
Grossing notes partial cystectomy
Site urachus, urinary bladder - specifically the dome
Signs +/-hematuria
Prevalence very rare
Prognosis usually poor
Clin. DDx other bladder tumours - esp. urothelial carcinoma

Urachal carcinoma is an uncommon malignant tumour that arises from the urachus. Most urachal carcinomas are adenocarcinomas.

General

  • Very rare[1]~ 0.2% of bladder cancers.[2]
  • Often younger <55 years-old.
  • Poor prognosis[3] - especially for high Sheldon stage.[4]

Clinical:

  • Hematuria - classic presenation.[3]

Treatment:

Gross

  • Lesion must be in urachus or dome of urinary bladder.

Microscopic

Features of epithelial malignancy:

  • Atypical (epithelial) cells.
  • Usually gland forming, i.e. adenocarcinoma.
  • +/-Mucinous component.

Features making it urothelial carcinoma (modified Sheldon criteria) - all required:[5]

  1. Located in bladder dome or anterior wall.
  2. Centered on bladder wall (as opposed to the mucosa).
  3. No widespread cystitis cystica/glandularis outside of the bladder dome and anterior wall.
  4. No known urothelial carcinoma elsewhere.

DDx:[6]

Patterns

  • Enteric - looks like colonic adenocarcinoma.
  • Mucinous.
  • Signet ring.

Note:

  • Urachal carcinoma may be nonglandular.[5]

Images

  • UC - very low mag. (WC)

  • UC - low mag. (WC)

  • UC - high mag. (WC)

IHC

Features:[4]

  • CK20 +ve.
  • CK7 +ve/-ve.
  • CK34betaE12 +ve/-ve.
  • Beta-catenin -- usu cytoplasmic/membranous +ve.

Others:[7]

  • p63 -ve (+ve in only 3%).

UC versus CRC -- not absolute but useful:

  • CK34betaE12 +ve in UC (-ve in CRC).
  • Beta-catenin -ve nuclei in UC (+ve nuclei in CRC).

Sign out

  • The diagnosis is a clinicopathologic diagnosis - one needs imaging to make it.[6]
  • May be staged with the Sheldon system.[2]

Sheldon staging system

Staging as Sheldon et al.:[4][8]

  • 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 nodes.
  • pT4b - metastasis to distant sites.

See also

References

  1. ↑ Ashley, RA.; Inman, BA.; Sebo, TJ.; Leibovich, BC.; Blute, ML.; Kwon, ED.; Zincke, H. (Aug 2006). "Urachal carcinoma: clinicopathologic features and long-term outcomes of an aggressive malignancy.". Cancer 107 (4): 712-20. doi:10.1002/cncr.22060. PMID 16826585.
  2. ↑ 2.0 2.1 Bruins, HM.; Visser, O.; Ploeg, M.; Hulsbergen-van de Kaa, CA.; Kiemeney, LA.; Witjes, JA. (Oct 2012). "The clinical epidemiology of urachal carcinoma: results of a large, population based study.". J Urol 188 (4): 1102-7. doi:10.1016/j.juro.2012.06.020. PMID 22901574.
  3. ↑ 3.0 3.1 Shao, GJ.; Cai, L.; Li, XS.; Song, G.; Li, XY.; He, ZS.; Zhou, LQ. (Oct 2013). "[Urachal carcinoma: experience of a clinical center within 30 years].". Beijing Da Xue Xue Bao 45 (5): 774-8. PMID 24136277.
  4. ↑ 4.0 4.1 4.2 Gopalan, A.; Sharp, DS.; Fine, SW.; Tickoo, SK.; Herr, HW.; Reuter, VE.; Olgac, S. (May 2009). "Urachal carcinoma: a clinicopathologic analysis of 24 cases with outcome correlation.". Am J Surg Pathol 33 (5): 659-68. doi:10.1097/PAS.0b013e31819aa4ae. PMID 19252435.
  5. ↑ 5.0 5.1 Paner, GP.; Barkan, GA.; Mehta, V.; Sirintrapun, SJ.; Tsuzuki, T.; Sebo, TJ.; Jimenez, RE. (Mar 2012). "Urachal carcinomas of the nonglandular type: salient features and considerations in pathologic diagnosis.". Am J Surg Pathol 36 (3): 432-42. doi:10.1097/PAS.0b013e31823fe49c. PMID 22301493.
  6. ↑ 6.0 6.1 Amin, Mahul B. (2010). Diagnostic Pathology: Genitourinary (1st ed.). Amirsys. pp. 2-143. ISBN 978-1931884280.
  7. ↑ Paner, GP.; McKenney, JK.; Barkan, GA.; Yao, JL.; Frankel, WL.; Sebo, TJ.; Shen, SS.; Jimenez, RE. (Jun 2011). "Immunohistochemical analysis in a morphologic spectrum of urachal epithelial neoplasms: diagnostic implications and pitfalls.". Am J Surg Pathol 35 (6): 787-98. doi:10.1097/PAS.0b013e3182189c11. PMID 21572312.
  8. ↑ Sheldon, CA.; Clayman, RV.; Gonzalez, R.; Williams, RD.; Fraley, EE. (Jan 1984). "Malignant urachal lesions.". J Urol 131 (1): 1-8. PMID 6361280.
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