Urachal cancer

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Urachal cancer
Urachal carcinoma - high mag.jpg
Micrograph of urachal carcinoma (right of image) and non-malignant urothelium (left of image). H&E stain.
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Urachal cancer is a very rare type of cancer arising from the urachus or its remnants. [1] The disease might arise from metaplastic glandular epithelium or embryonic epithelial remnants originating from the cloaca region. [2]

Contents

It occurs in roughly about one person per 1 million people per year varying on the geographical region. [3] Men are affected slightly more often than women mostly in the 5th decade of life but the disease can occur in also in other age groups. [4] [5]

It can involve the urinary bladder, but is not bladder cancer in the usual sense. Urachal cancer can occur at any site along the urachal tract.

Urachal cancer was mentioned by Hue and Jacquin in 1863 followed by an elaborate work by T. Cullen in 1916 about diseases of the umbilicus, while C. Begg further characterized urachal cancer in the 1930s. [2] [6] Detailed diagnostic and staging schemes were proposed by Sheldon et al. in 1984, which remain widely used today. [1]

Classification

Urachal cancer usually is an adenocarcinoma (about 90%) mostly with mucinous/colloidal histology. Other rare types include urothelial carcinoma, squamous cell carcinoma, neuroendocrine carcinoma and sarcoma. [2] [4] [7] [8]

Symptoms and Signs

Urachal cancer can exist for some years without any symptoms. The most frequent initial symptom is haematuria which occurs when the urachal tumour has penetrated the bladder wall, but mucinuria (mucin in the urine), local pain or swelling, recurrent local or urinary tract infections and umbilical discharge can (but is not always) be seen. [4] [5]

Causes

Mechanism

Diagnosis

According to the American Urological Association, the diagnostic criteria are the following: [9]

  1. The location of the tumor is mostly at the bladder dome.
  2. No findings of cystitis glandularis on the bladder surface. These findings can be precursor lesions of a primary bladder adenocarcinoma.
  3. No history of a different primary adenocarcinoma with the same morphology.

Tissue for histological analysis is usually obtained via a transurethral resection of bladder tumor (TURBT).

Histopathological Diagnosis

A case of urachal adenocarcinoma demonstrating immunohistochemical CDX2-positivity with typical nuclear staining. 200x magnification. UrC CDX2 200x.tif
A case of urachal adenocarcinoma demonstrating immunohistochemical CDX2-positivity with typical nuclear staining. 200x magnification.

According to the current version (4th ed.) of the WHO classification of Tumours of the Urinary System, the diagnosis of a urachal adenocarcinoma usually needs a location of the tumor in the bladder dome and/or anterior wall, epicenter of the tumor in the bladder wall, absence of widespread cystitis cystica and/or cystitis glandularis beyond the dome or anterior wall, and absence of another similar structured tumor as a primary tumor elsewhere in the body. A urachal remnant in association with the tumor can help with the diagnosis. [10] [11] [12] [13] In addition, other diagnostic systems also for urachal carcinomas that are not adenocarcinomas have been proposed. [14]

Immunohistochemical stains are usually less helpful in the histopathological differential diagnosis of urachal adenocarcinomas. [15]

Imaging

CT and MRI scans are useful to evaluate local invasion and metastasis to lymph nodes and other parts of the body. Besides, in 32 to 46% of the cases, they show calcifications which is very suggestive of the disease. [16]

Treatment

Surgical management is en bloc resection of bladder, urachal remnant, and umbilicus. [9] In progressed stages, radiotherapy seems not to lead to sufficient response rates. However, chemotherapy regimes containing 5-FU (and Cisplatin) have been described to be useful in these cases. [4] [17] In recent years, targeted therapies have been demonstrated to be useful in reports of single cases. These agents included Sunitinib, [18] Gefitinib, [19] Bevacizumab [20] and Cetuximab. [21]

Serum Markers for Monitoring

Measurement of serum concentrations of CEA, CA19-9 and CA125 can be helpful in monitoring urachal cancer [2] [22]

Outcomes

The 5-year survival is estimated between 25 and 61%. [9] Worse prognostic factors include the presence of residual tumor at the margin of the resection specimen (R+), invasion of the peritoneum and metastatic disease. [4]

References

  1. 1 2 Sheldon, C. A.; Clayman, R. V.; Gonzalez, R.; Williams, R. D.; Fraley, E. E. (1984-01-01). "Malignant urachal lesions". The Journal of Urology. 131 (1): 1–8. doi:10.1016/s0022-5347(17)50167-6. ISSN   0022-5347. PMID   6361280.
  2. 1 2 3 4 Paner, Gladell P.; Lopez-Beltran, Antonio; Sirohi, Deepika; Amin, Mahul B. (2016-03-01). "Updates in the Pathologic Diagnosis and Classification of Epithelial Neoplasms of Urachal Origin". Advances in Anatomic Pathology. 23 (2): 71–83. doi:10.1097/PAP.0000000000000110. ISSN   1533-4031. PMID   26849813. S2CID   35894913.
  3. ↑ Bruins, H. Max; Visser, Otto; Ploeg, Martine; Hulsbergen-van de Kaa, Christina A.; Kiemeney, Lambertus A. L. M.; Witjes, J. Alfred (2012-10-01). "The clinical epidemiology of urachal carcinoma: results of a large, population based study". The Journal of Urology. 188 (4): 1102–1107. doi:10.1016/j.juro.2012.06.020. ISSN   1527-3792. PMID   22901574.
  4. 1 2 3 4 5 Szarvas, Tibor; Módos, Orsolya; Niedworok, Christian; Reis, Henning; Szendröi, Attila; Szász, Marcell A.; Nyirády, Péter (2016-06-03). "Clinical, prognostic, and therapeutic aspects of urachal carcinoma-A comprehensive review with meta-analysis of 1,010 cases". Urologic Oncology. 34 (9): 388–398. doi:10.1016/j.urolonc.2016.04.012. ISSN   1873-2496. PMID   27267737.
  5. 1 2 Behrendt, Mark A.; DE Jong, Jeroen; VAN Rhijn, Bas W. (2016-04-01). "Urachal cancer: contemporary review of the pathological, surgical, and prognostic aspects of this rare disease". Minerva Urologica e Nefrologica. 68 (2): 172–184. ISSN   0393-2249. PMID   26583595.
  6. ↑ Begg, RC (1931). "The colloid adenocarcinomata of the bladder vault arising from the epithelium of the urachal canal: with a critical survey of the tumours of the urachus". Br J Surg. 18 (71): 422–466. doi:10.1002/bjs.1800187108. S2CID   70712744.
  7. ↑ Wright, Jonathan L.; Porter, Michael P.; Li, Christopher I.; Lange, Paul H.; Lin, Daniel W. (2006-08-15). "Differences in survival among patients with urachal and nonurachal adenocarcinomas of the bladder". Cancer. 107 (4): 721–728. doi:10.1002/cncr.22059. ISSN   0008-543X. PMID   16826584.
  8. ↑ Reis, Henning; Krafft, Ulrich; Niedworok, Christian; Módos, Orsolya; Herold, Thomas; Behrendt, Mark; Al-Ahmadie, Hikmat; Hadaschik, Boris; Nyirady, Peter; Szarvas, Tibor (2018). "Biomarkers in Urachal Cancer and Adenocarcinomas in the Bladder: A Comprehensive Review Supplemented by Own Data". Disease Markers. 2018: 7308168. doi: 10.1155/2018/7308168 . ISSN   1875-8630. PMC   5867586 . PMID   29721106.
  9. 1 2 3 "Urachal Adenocarcinoma". AUAnet.org.
  10. ↑ Humphrey, Peter A.; Moch, Holger; Cubilla, Antonio L.; Ulbright, Thomas M.; Reuter, Victor E. (July 2016). "The 2016 WHO Classification of Tumours of the Urinary System and Male Genital Organs—Part B: Prostate and Bladder Tumours". European Urology. 70 (1): 106–119. doi:10.1016/j.eururo.2016.02.028. ISSN   0302-2838. PMID   26996659.
  11. ↑ Gopalan, Anuradha; Sharp, David S.; Fine, Samson W.; Tickoo, Satish K.; Herr, Harry W.; Reuter, Victor E.; Olgac, Semra (May 2009). "Urachal Carcinoma". American Journal of Surgical Pathology. 33 (5): 659–668. doi:10.1097/pas.0b013e31819aa4ae. ISSN   0147-5185. PMC   4225778 . PMID   19252435.
  12. ↑ Herr, Harry W.; Bochner, Bernard H.; Sharp, David; Dalbagni, Guido; Reuter, Victor E. (July 2007). "Urachal Carcinoma: Contemporary Surgical Outcomes" . Journal of Urology. 178 (1): 74–78. doi:10.1016/j.juro.2007.03.022. ISSN   0022-5347. PMID   17499279.
  13. ↑ Sheldon, Curtis A.; Clayman, Ralph V.; Gonzalez, Ricardo; Williams, Richard D.; Fraley, Elwin E. (January 1984). "Malignant Urachal Lesions" . Journal of Urology. 131 (1): 1–8. doi:10.1016/s0022-5347(17)50167-6. ISSN   0022-5347. PMID   6361280.
  14. ↑ Paner, Gladell P.; Barkan, Güliz A.; Mehta, Vikas; Sirintrapun, Sahussapont Joseph; Tsuzuki, Toyonori; Sebo, Thomas J.; Jimenez, Rafael E. (March 2012). "Urachal carcinomas of the nonglandular type: salient features and considerations in pathologic diagnosis". The American Journal of Surgical Pathology. 36 (3): 432–442. doi:10.1097/PAS.0b013e31823fe49c. ISSN   1532-0979. PMID   22301493. S2CID   5277019.
  15. ↑ Reis, H.; Szarvas, T. (June 2019). "Urachal cancer-current concepts of a rare cancer". Der Pathologe. 40 (Suppl 1): 31–39. doi:10.1007/s00292-018-0516-9. ISSN   1432-1963. PMID   30895340. S2CID   84185828.
  16. ↑ Claps, Mélanie; Stellato, Marco; Zattarin, Emma; Mennitto, Alessia; Sepe, Pierangela; Guadalupi, Valentina; Mennitto, Roberta; de Braud, Filippo G.M.; Verzoni, Elena; Procopio, Giuseppe (January 2020). "Current Understanding of Urachal Adenocarcinoma and Management Strategy" . Current Oncology Reports. 22 (1): 9. doi:10.1007/s11912-020-0878-z. ISSN   1523-3790. PMID   31989430. S2CID   210925794.
  17. ↑ Siefker-Radtke, Arlene (2012-10-01). "Urachal adenocarcinoma: a clinician's guide for treatment". Seminars in Oncology. 39 (5): 619–624. doi:10.1053/j.seminoncol.2012.08.011. ISSN   1532-8708. PMID   23040259.
  18. ↑ Testa, Isabella; Verzoni, Elena; Grassi, Paolo; Colecchia, Maurizio; Panzone, Filomena; Procopio, Giuseppe (2014-10-27). "Response to targeted therapy in urachal adenocarcinoma". Rare Tumors. 6 (4): 5529. doi:10.4081/rt.2014.5529. ISSN   2036-3605. PMC   4274441 . PMID   25568747.
  19. ↑ Goss, G.; Hirte, H.; Miller, W. H.; Lorimer, I. a. J.; Stewart, D.; Batist, G.; Parolin, D. a. E.; Hanna, P.; Stafford, S. (2005-03-01). "A phase I study of oral ZD 1839 given daily in patients with solid tumors: IND.122, a study of the Investigational New Drug Program of the National Cancer Institute of Canada Clinical Trials Group". Investigational New Drugs. 23 (2): 147–155. doi:10.1007/s10637-005-5860-y. ISSN   0167-6997. PMID   15744591. S2CID   20979000.
  20. ↑ Kanamaru, Tomohiro; Iguchi, Taro; Yukimatsu, Nao; Shimizu, Yasuomi; Kohyama, Yuki; Tachibana, Hirokazu; Kato, Minoru; Yamasaki, Takeshi; Tamada, Satoshi (2015-03-01). "A Case of Metastatic Urachal Carcinoma Treated With FOLFIRI (irinotecan and 5-Fluorouracil/leucovorin) Plus Bevacizumab". Urology Case Reports. 3 (2): 9–11. doi:10.1016/j.eucr.2014.11.004. ISSN   2214-4420. PMC   4714276 . PMID   26793485.
  21. ↑ Collazo-Lorduy, Ana; Castillo-Martin, Mireia; Wang, Li; Patel, Vaibhav; Iyer, Gopa; Jordan, Emmet; Al-Ahmadie, Hikmat; Leonard, Issa; Oh, William K. (2016-05-10). "Urachal Carcinoma Shares Genomic Alterations with Colorectal Carcinoma and May Respond to Epidermal Growth Factor Inhibition". European Urology. 70 (5): 771–775. doi:10.1016/j.eururo.2016.04.037. ISSN   1873-7560. PMC   5489411 . PMID   27178450.
  22. ↑ Siefker-Radtke, Arlene O.; Gee, Jason; Shen, Yu; Wen, Sijin; Daliani, Danai; Millikan, Randall E.; Pisters, Louis L. (2003-04-01). "Multimodality management of urachal carcinoma: the M. D. Anderson Cancer Center experience". The Journal of Urology. 169 (4): 1295–1298. doi:10.1097/01.ju.0000054646.49381.01. ISSN   0022-5347. PMID   12629346.
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