Urachal carcinoma

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

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

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 presentation.[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 urachal 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.

Notes:

  • Gopalan et al.[4] (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:[6]

Patterns

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

Note:

  • Urachal carcinoma may be nonglandular.[5]

Images

  • UC - very low mag. (WC/Nephron)

  • UC - low mag. (WC/Nephron)

  • UC - intermed mag. (WC/Nephron)

  • UC - high mag. (WC/Nephron)

  • UC - very high mag. (WC/Nephron)

IHC

Features:[4]

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

Others:[8]

  • 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 per Sheldon et al.:[4][9]

  • 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 4.3 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. ↑ Joniau, S.; Lerut, E.; Van Poppel, H. (2009). "A Giant Mucinous Adenocarcinoma Arising within a Villous Adenoma of the Urachus: Case Report and Review of the Literature.". Case Rep Med 2009: 818646. doi:10.1155/2009/818646. PMID 20182635.
  8. ↑ 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.
  9. ↑ 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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