Urachal cancer

Urachal cancer is a very rare type of cancer arising from the urachus or its remnants.[1] The disease might arise from metaplasic glandular epithelium or embryonic epithelial remnants originating from the cloaca region.[2]

Urachal cancer
Micrograph of urachal carcinoma (right of image) and non-malignant urothelium (left of image). H&E stain.
SpecialtyOncology 

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]

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]

Diagnosis

According to the American Urological Association, the diagnostic criteria are the following:[8]

  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.

According to the American Urological Association, the histologic findings are the following:[8]

  1. Mucinous carcinoma (tumor cells within mucin microcollections)
  2. Enteric morphology (i.e. colorectal adenocarcinoma),
  3. Other morphologies: signet ring cell, adenocarcinoma, mixed, or unclassifiable.
  4. Immunohistochemical staining (CDX2+, CK7+, β-catenin-)

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.[9]

Treatment

Surgical management is en bloc resection of bladder, urachal remnant, and umbilicus.[8] 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][10] In recent years, targeted therapies have been demonstrated to be useful in reports of single cases. These agents included Sunitinib,[11] Gefitinib,[12] Bevacizumab[13] and Cetuximab.[14]

Serum Markers for Monitoring

Measurement of serum concentrations of CEA, CA19-9 and CA125 can be helpful in monitoring urachal cancer[2][15]

Outcomes

The 5-year survival is estimated between 25 and 61%.[8] 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. 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. 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.
  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. 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. 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.
  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. "Urachal Adenocarcinoma". AUAnet.org.
  9. 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.
  10. 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.
  11. 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.
  12. 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.
  13. 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.
  14. 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.
  15. 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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