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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 26  |  Issue : 2  |  Page : 225-226

Urachal adenocarcinoma – A rare case report in a young female


Department of Urology, Medical College, Thiruvananthapuram, Kerala, India

Date of Submission28-Jul-2020
Date of Decision13-Aug-2020
Date of Acceptance05-Oct-2020
Date of Web Publication07-Nov-2020

Correspondence Address:
Dr. Anil Sundaram
Department of General Surgery, Medical College, Thiruvananthapuram - 695 011, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ksj.ksj_3_20

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  Abstract 


Urachal adenocarcinoma has come to the limelight of surgical practice during the last three decades. Due to the rarity of this tumour, the complete biological behaviour and treatment protocols have not been developed. This is a case report of a 22-year-old female who was diagnosed with urachal adenocarcinoma and underwent radical surgery with adjuvant chemotherapy based on a multidisciplinary team meeting. This is the youngest case reported from India.

Keywords: Allantois, immunohistochemistry, urachal adenocarcinoma


How to cite this article:
Sundaram A, Kumar M, Kurup S. Urachal adenocarcinoma – A rare case report in a young female. Kerala Surg J 2020;26:225-6

How to cite this URL:
Sundaram A, Kumar M, Kurup S. Urachal adenocarcinoma – A rare case report in a young female. Kerala Surg J [serial online] 2020 [cited 2020 Nov 30];26:225-6. Available from: http://www.keralasurgj.com/text.asp?2020/26/2/225/300252




  Introduction Top


Urachal carcinoma was first described in 1963 by Hue and Jacquin.[1] Urachal adenocarcinoma develops from malignant transformation of columnar metaplastic epithelium of the embryological remnant of the allantois. Following the descent of the bladder into the pelvis during the 4th month of foetal development, urachus is stretched until it becomes the median umbilical ligament that joins the umbilicus to the dome of the bladder. Urachal carcinoma constitutes less than 0.5% of bladder malignancies.[2] Among this, adenocarcinoma constitutes 85%–90%.[2] The youngest adult (22 years old) case of urachal cancer was reported by Siefker-Radtke et al. from MD Anderson Cancer Center (MDACC).


  Case Report Top


A 22-year-old female presented to the outpatient department with symptoms of dysuria and lower abdomen pain. Abdomen examination revealed an 8 cm × 6 cm intra-abdominal mass palpable in hypogastrium and umbilical area with inferior border not palpable, irregular surface and hard in consistency. Ultrasound showed an 8 cm × 7 cm intraperitoneal lesion with both hypo- and hyperechoic areas arising from the dome of the bladder. Contrast-enhanced computed tomography abdomen showed an 8 cm × 7 cm × 6 cm intraperitoneal lesion with both cystic and solid areas arising from the dome of the bladder into space of Retzius and extending intraperitoneally with no invasion of adjacent bowel loops and reaching 1 cm below the level of umbilicus with stippled calcification suggesting an urachal adenocarcinoma [Figure 1]a. Magnetic resonance imaging (MRI) abdomen was done to rule out anterior abdominal wall involvement and to assess the pelvic nodes. An enlarged 1 cm × 1 cm iliac group of lymph nodes was present in MRI [Figure 1]b. Cystoscopy was done which showed a polypoidal area projecting into bladder with gelatinous discharge and biopsy was taken. She underwent partial cystectomy + pelvic lymph node dissection + Resection of the umbilicus [Figure 2]a and [Figure 2]b. Histopathology report was urachal mucinous cystadenocarcinoma moderately differentiated [Figure 3]a. Caudal-type homeobox 2 (CDX2) was positive [Figure 3]b and cytokeratin (CK) 20 was also positive [Figure 3]c was also positive. The anterior abdominal wall and umbilicus were free of neoplasm. There was no metastasis to pelvic lymph nodes. She was placed on six cycles of cisplatin + 5-fluorouracil-based chemotherapy.
Figure 1: (a) Contrast-enhanced computed tomography – Stippled calcification. (b) Magnetic resonance imaging – Lymph node involvement

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Figure 2: (a) Excised gross specimen. (b) Lesion protruding into the bladder lumen

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Figure 3: (a) H and E staining. (b) Caudal-type homeobox 2. (c) Cytokeratin 20

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  Discussion Top


Malignancy in the urachus is reported in one in five million people.[3] Till now, in literature, only 7 cases of urachal adenocarcinoma have been reported in less than 30 years of age. Sheldon proposed a staging system as follows: pT1, no invasion beyond the urachal mucosa; pT2, invasion confined to the urachus; pT3, local extension to the (a) bladder, (b) abdominal wall and (c) viscera other than the bladder; and pT4, metastasis to (a) regional lymph nodes and (b) distant sites.[1] The diagnosis of urachal cancers has been made easier by the MDACC criteria consisting of two main criteria and four supportive criteria. The two main criteria consist of midline location of the tumour and a sharp demarcation between the tumour and normal surface epithelium. Supportive criteria include an enteric histology, the absence of urothelial dysplasia, the absence of cystitis cystica and the absence of a primary adenocarcinoma of another origin.[4] Haematuria is the most common symptom (71%), pain (42%), irritative symptoms (40%) and umbilical discharge (2%). Suprapubic mass and mucinuria occur in about 25%.[5]

Most cases of urachal adenocarcinoma occur in the fifth and sixth decades. Global 5-year survival is approximately 49% after urachal adenocarcinoma treatment. An en bloc resection of the urachal ligament and umbilicus is recommended for patients who have surgically resectable disease, along with either a complete or partial cystectomy, to ensure negative margins Radiologically if the nodes are positive pelvic lymph node dissection must be done.[6]

Immunohistochemistry plays a pivotal role in differentiating primary urachal adenocarcinoma from secondary ones. β-catenin, CDX2, CK7 and CK20 are the commonly used markers. In our case, diffuse nuclear β-catenin was negative, and CK7, CDX2 and CK20 were positive. These helped to differentiate urachal adenocarcinoma of enteric subtype from colonic adenocarcinoma, which are β-catenin positive and CK7 negative.

Personalised chemotherapy may be a curative option in those patients with moderate or poorly differentiated tumours as in this case.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Sheldon CA, Clayman RV, Gonzalez R, Williams RD, Fraley EE. Malignant urachal lesions. J Urol 1984;131:1-8.  Back to cited text no. 1
    
2.
Siefker-Radtke AO, Gee J, Shen Y, Wen S, Daliani D, Millikan RE, et al. Multimodality management of urachal carcinoma: The M. D. Anderson Cancer Center experience. J Urol 2003;169:1295-8.  Back to cited text no. 2
    
3.
Pinthus JH, Haddad R, Trachtenberg J, Holowaty E, Bowler J, Herzenberg AM, et al. Population based survival data on urachal tumors. J Urol 2006;175:2042-7.  Back to cited text no. 3
    
4.
Gopalan A, Sharp DS, Fine SW, Tickoo SK, Herr HW, Reuter VE, et al. Urachal carcinoma: A clinicopathologic analysis of 24 cases with outcome correlation. Am J Surg Pathol 2009;33:659-68.  Back to cited text no. 4
    
5.
Tsiouris A, Ahmed HU, Kumar N, Kaisary AV. Urachal tumour: Clinical and radiological features of a poorly understood carcinoma. Ann R Coll Surg Engl 2007;89:17-8.  Back to cited text no. 5
    
6.
Osunkoya AO, Epstein JI. Primary mucin-producing urothelial-type adenocarcinoma of prostate: Report of 15 cases. Am J Surg Pathol 2007;31:1323-9.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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