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Table of Contents
CASE REPORT
Year : 2020  |  Volume : 12  |  Issue : 3  |  Page : 291-294  

Mucinous cystadenomas of urachus: A case report and literature review


1 Department of Pathophysiology, American University of Antigua College of Medicine, Antigua, Antigua and Barbuda, India
2 Department of Surgical Gastroenterology, Nishtha Surgical Hospital and Research Centre, Patan, India
3 Consultant Pathologist, Neberg Supratech Laboratory, Mehsana, Gujarat, India

Date of Submission27-Aug-2019
Date of Acceptance19-Jun-2020
Date of Web Publication17-Jul-2020

Correspondence Address:
Dr. Vipul D Yagnik
77, Siddhraj Nagar, Rajmahal Road, Patan - 384 265, Gujrat
India
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DOI: 10.4103/UA.UA_118_19

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   Abstract 


Urachal epithelial neoplasms are rare tumors that arise from the vestiges of the urachus. Mucinous cystadenomas are considered as a benign glandular tumors of the urachus. Cystadenomas are commonly found in the ovary, appendix, and pancreas. Mucinous cystadenomas of the urachus are extremely rare in the urachus, and only nine cases reported so far. We reported the 10th case of Mucinous cystadenomas of the urachus detected incidentally at the time of diagnostic laparoscopy for investigation of genitourinary tuberculosis.

Keywords: Cystic neoplasm, mucinous cystadenomas, urachus


How to cite this article:
Agnihotri AK, Yagnik VD, Agnihotri S, Yagnik B. Mucinous cystadenomas of urachus: A case report and literature review. Urol Ann 2020;12:291-4

How to cite this URL:
Agnihotri AK, Yagnik VD, Agnihotri S, Yagnik B. Mucinous cystadenomas of urachus: A case report and literature review. Urol Ann [serial online] 2020 [cited 2020 Aug 12];12:291-4. Available from: http://www.urologyannals.com/text.asp?2020/12/3/291/289955




   Introduction Top


Mucinous cystadenomas are benign cystic neoplasms filled with mucin and lined by well-differentiated columnar mucinous epithelium.[1] These tumors are mainly found in the ovaries and rarely occur in children and adolescents.[2] Other common sites of mucinous cystadenomas are the appendix and pancreas.[3],[4],[5] An English literature review shows only nine reported cases of urachal mucinous cystadenoma so far.[6],[7],[8],[9],[10],[11] Mucinous cystadenomas are covered under an updated 2016 World Health Organization classification of genitourinary tumors.[12] Hull and Warfel reported the first time a case of a mucinous cystadenoma as an incidental finding in the year 1994.[6] Later on, Paul et al. described the first case of urachal mucinous adenocarcinoma at the stage of carcinoma in situ and suggested a premalignant change in the urachal remnant.[13]

Urachus usually obliterates to a fibrous band, known as the median umbilical ligament before birth. However, incomplete closure may lead to various pathologies such as cysts, diverticulum, fistula, infections, and neoplasms.[14] Among them, neoplasms are less reported and need more attention because of the ability of benign neoplasms to turn into malignant ones. These neoplasms can be broadly categorized into noncystic frankly invasive and mucinous cystic tumors on the bases of overall architecture.[12] The mucinous cystic tumors are further subdivided according to the scheme used for mucinous ovarian tumors such as mucinous cystadenoma – a single layer of mucinous columnar epithelium with no atypia, mucinous cystic tumor of low malignant potential (MCTLMP) – epithelial proliferation including formation of papillae with low-grade cytologic atypia, and mucinous cystadenocarcinoma – malignant cytologic features with microinvasion or frank invasion.[15] We attempt to share a case of mucinous cystadenoma from the aspects of clinical presentation, operative findings, and histopathology because of its rare entity, aggressive behavior to develop into pseudomyxoma peritonei, and the possibility of malignant transformation.


   Case Details Top


A 27-year-old female presented to the surgical outpatient department with chief complaints of lower abdominal pain and vomiting for 7–8 days. She also reported a history of diarrhea a few days back. She had malaise, weakness, evening rise of temperature, and weight loss. On physical examination, the abdomen was distended but soft and not tender. Her vitals were stable. Her respiratory and cardiovascular system examinations were unremarkable. Her menstrual cycles were regular. Ultrasonography of the abdomen and contrast-enhanced computed tomography scan were suggestive of abdominal tuberculosis predominantly genitourinary tuberculosis. Laboratory examination showed hemoglobin of 8 g/dl, a white blood cell count of 12,370/μL, platelet count of 455,000/μL of blood, and normal renal and liver profile. She was not diabetic and hypertensive. Her viral serology for the human immunodeficiency virus was nonreactive.

A diagnostic laparoscopy revealed a frozen pelvis and incidentally found a well-defined rounded swelling in the suprapubic region in the line of median umbilical ligament at the suprapubic region [Figure 1]. An excisional biopsy demonstrated a well-circumscribed grayish multilocular cystic lesion filled with gelatinous material measuring 2.5 cm × 2.5 cm × 2 cm [Figure 2]a. Histologically, sections revealed a cystic nodule lined by mucin-secreting columnar epithelium with basal nuclei, abundant apical mucin, and focal pseudostratification, but no nuclear atypia was seen [Figure 2]b. The cystic wall was composed of fibrocollagenous, muscular wall, and adipose tissue, and the lumen contained proteinaceous and myxoid materials with calcification. A diagnosis of urachus mucinous cystadenoma was confirmed. The postoperative course was unpretentious, and the patient was discharged on the 4th postoperative day. The patient is in follow-up without any complaints.
Figure 1: Well-defined rounded swelling in the suprapubic region in the line with median umbilical ligament

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Figure 2: (a) Well-circumscribed grayish multilocular cystic lesion filled with gelatinous material, (b) mucin-secreting columnar epithelium with basal nuclei, abundant apical mucin, and pseudostratification

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   Discussion Top


Urachal masses are relatively uncommon in clinical practice and remain undiagnosed for an extended period because of their obscured location. These lesions are often identified as an incidental finding or at an advanced stage of malignancy. The differential diagnosis includes both neoplastic and nonneoplastic conditions. Mucinous cystadenomas represent a small percentage of urachal mucinous cystic neoplasms with a possibility of malignant transformation.[5],[9] [Table 1] summarizes the demographic characteristics, clinical presentations, and histopathologic features of mucinous cystadenomas in order to differentiate them from a MCTLMP and mucinous cystadenocarcinoma.
Table 1: Clinicopathologic characteristics of urachal mucinous cystadenoma

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Urachal remnants may persist in approximately 32% of adults.[16] Although the origin and pathophysiology of urachal tumors are not fully understood; It is believed that the malignant transformation of the columnar or glandular metaplastic epithelium of the urachus results in urachal carcinoma.[17] Prophylactic excision of benign urachal lesions has been recommended because the presence of urachal remnant predisposes to malignancy.[7]

Urachal mucinous cystadenoma is a rarely reported neoplasm and can be presented as lower abdominal mass,[7],[9] lower abdominal pain,[9],[10] urinary frequency,[8] and sometimes hematuria.[9] They have the potential to rupture and develop into pseudomyxoma peritonei.[9] Histology described in this case report confirms the diagnosis of mucinous cystadenoma that lacked the evidence of nuclear atypia and invasion. To our knowledge, this is the 10th reported case of urachal mucinous cystadenoma in the English literature.


   Conclusion Top


Although rare, urachal mucinous cystadenomas should be included in the differential diagnosis of suprapubic lower abdominal mass. The patient should be followed clinically after excision for the evidence of local recurrence.

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.
Kumar V, Abbas AK, Aster Jon C. The female genital tract. In: Robbins and Cotran Pathologic Basis of Disease. 9th ed. Philadelphia, PA: Elsevier/Saunders; 2015.  Back to cited text no. 1
    
2.
Cowan RA, Haber EN, Faucz FR, Stratakis CA, Gomez-Lobo V. mucinous cystadenoma in children and adolescents. J Pediatr Adolesc Gynecol 2017;30:495-8.  Back to cited text no. 2
    
3.
Janczak D, Szponder M, Janczak D, Leśniak M, Ziomek A, Chabowski M. Mucinous cystadenoma of the appendix - case report. Pol Przegl Chir 2017;89:65-8.  Back to cited text no. 3
    
4.
Dorobanţu BM, Matei E, Herlea V, Boroş M, Tivadar B, Ciurea SH. Diagnosis, morphopathological profile and treatment of mucinous cystadenoma of the pancreas – A single center experience. Rom J Morphol Embryol 2018;59:1155-63.  Back to cited text no. 4
    
5.
Wang D, Sule N. Mucinous cystadenoma of the urachus and review of current classification of urachal mucinous cystic neoplasms. Arch Pathol Lab Med 2019;143:258-63.  Back to cited text no. 5
    
6.
Hull MT, Warfel KA. Urachal cystadenoma with abundant glycogen: Ultrastructural study. Ultrastruct Pathol 1994;18:499-502.  Back to cited text no. 6
    
7.
Schell AJ, Nickel CJ, Isotalo PA. Complex mucinous cystadenoma of undetermined malignant potential of the urachus. Can Urol Assoc J 2009;3:E39-E41.  Back to cited text no. 7
    
8.
Saha S, Jha A, Gupta S. Urachal mucinous cystadenoma. Saudi J Kidney Dis Transpl 2011;22:1256-8.  Back to cited text no. 8
[PUBMED]  [Full text]  
9.
Amin MB, Smith SC, Eble JN, Rao P, Choi WW, Tamboli P, et al. Glandular neoplasms of the urachus: A report of 55 cases emphasizing mucinous cystic tumors with proposed classification. Am J Surg Pathol 2014;38:1033-45.  Back to cited text no. 9
    
10.
Gupta S, Bhaijee F, Harmon EP. Mucinous neoplasm arising in a urachal cyst: A first in the pediatric population. Urology 2014;83:455-6.  Back to cited text no. 10
    
11.
Wang D, Kauffman E, Sule N. Mucinous cystadenoma of the urachus: A case report and review of the literature. Am J Clin Pathol 2016;146:57.  Back to cited text no. 11
    
12.
Paner GP, Lopez-Beltran A, Sirohi D, Amin MB. Updates in the pathologic diagnosis and classification of epithelial neoplasms of urachal origin. Adv Anat Pathol 2016;23:71-83.  Back to cited text no. 12
    
13.
Paul AB, Hunt CR, Harney JM, Jenkins JP, McMahon RF. Stage 0 mucinous adenocarcinoma in situ of the urachus. J Clin Pathol 1998;51:483-4.  Back to cited text no. 13
    
14.
Naiditch JA, Radhakrishnan J, Chin AC. Current diagnosis and management of urachal remnants. J Pediatr Surg 2013;48:2148-52.  Back to cited text no. 14
    
15.
Scully RE, Young RH, Clement PB. Tumors of the Ovary, Maldeveloped Gonads, Fallopian Tube, and Broad Ligament. Washington, D.C: Armed Forces Institute of Pathology Available from the American Registry of Pathology, Armed Forces Institute of Pathology; 1998.  Back to cited text no. 15
    
16.
Schubert GE, Pavkovic MB, Bethke-Bedürftig BA. Tubular urachal remnants in adult bladders. J Urol 1982;127:40-2.  Back to cited text no. 16
    
17.
Sheldon CA, Clayman RV, Gonzalez R, Williams RD, Fraley EE. Malignant urachal lesions. J Urol 1984;131:1-8.  Back to cited text no. 17
    


    Figures

  [Figure 1], [Figure 2]
 
 
    Tables

  [Table 1]



 

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