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CASE REPORT
Year : 2011  |  Volume : 7  |  Issue : 2  |  Page : 223-225

Urachal papillary cystadenocarcinoma: A rare case report


Department of Pathology, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India

Date of Web Publication12-Jul-2011

Correspondence Address:
Kavita Mardi
12-A, Type V Quarters, GAD Colony, Kasumpti, Shimla, Himachal Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.82934

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 > Abstract 

Urachal papillary mucinous cystadenocarcinoma is a rare tumor and represents 0.17-0.34% of all bladder tumors. It has an insidious course and variable clinical presentation. We present a case report of a 37-year-old female who presented with a lump in the abdomen. MRI revealed a solidcystic mass in the right lumbar region. Fine needle aspiration cytology was suggestive of adenocarcinoma. Histopathological examination of the excised mass revealed papillary adenocarcinoma that brought out the presence of focal PAS-positive intracytoplasmic mucin in the tumor cells. Clinicians should have a high degree of suspicion for these rare tumors.

Keywords: Papillary adenocarcinoma, urachal remnants, urinary bladder


How to cite this article:
Mardi K, Gupta N. Urachal papillary cystadenocarcinoma: A rare case report. J Can Res Ther 2011;7:223-5

How to cite this URL:
Mardi K, Gupta N. Urachal papillary cystadenocarcinoma: A rare case report. J Can Res Ther [serial online] 2011 [cited 2019 Sep 21];7:223-5. Available from: http://www.cancerjournal.net/text.asp?2011/7/2/223/82934


 > Introduction Top


The urachus is a vestigial structure that connects the bladder to the allantois during early embryonic development. It remains patent in a small proportion of adults and becomes clinically important when only complicated by infection, cystic dilation, or when neoplasia develops in it. Urachal adenocarcinomas are extremely rare and their accurate preoperative diagnosis needs a high degree of clinical suspicion and correlation with the radiological findings. We herein report a case of papillary cystadenocarcinoma arising from the urachal tract in a 37-year-old female, to increase awareness regarding this rare entity.


 > Case Report Top


A 37-year-old female presented with complaints of a lump in the abdomen since 1 month. On examination, a firm-to-hard, immobile lump in the right lumbar region measuring 5 × 5 cm was found. Postcontrast MRI revealed a cystic mass with a solid component in the right lumbar region. The cystic mass showed enhancement of the wall and the solid part [Figure 1]. On fine needle aspiration (FNA) of this lump, the fluid was aspirated and smears prepared from the sediment revealed papillary fragments of tumor cells with a hyperchromatic nucleus, variably prominent nucleoli, and moderate amounts of basophilic cytoplasm. Serum CA-125 levels were also within normal limits. The patient underwent exploratory laparotomy. Peroperatively, a well-circumscribed and irregular mass measuring 6 × 5 cm originated from the urachal tract which was extending up to the dome of the bladder. The mass was excised.
Figure 1: Postcontrast MRI revealing a cystic mass with a solid component in the right lumbar region, showing enhancement of the wall and solid part

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On gross examination, a fat-laden soft tissue mass measuring 7 × 6 × 5 cm was revealed. The cut Surface of the mass was partly solid and partly cystic. The solid portion was revealing papillary excrescences [Figure 2]. The cystic portion was thick walled with variably sized solid nodules and focal papillary excrescences on the inner surface. Microscopic examination revealed pleomorphic tumor cells arranged in papillae, acini, labrynthine pattern, and solid sheets, separated by thick fibrous septae. These pleomorphic tumor cells showed a vesicular to hyperchromatic nucleus with prominent eosinophilic nucleoli [Figure 3] and [Figure 4]. Numerous bizarre mitotic figures were seen. In addition, there were extensive areas of hemorrhage and necrosis. The PAS stain revealed focal intracytoplasmic mucin in some of the tumor cells. The diagnosis of papillary mucinous cystadenocarcinoma arising from the patent urachus was rendered.
Figure 2: FNA smears revealing papillary fragment of tumor cells (Giemsa, ×40)

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Figure 3: Gross photograph showing a partly solid and partly cystic tumor revealing solid nodules and papillary excrescences on the inner surface

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Figure 4: Photomicrograph showing papillae lined by pleomorphic tumor cells (H and E, ×40)

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 > Discussion Top


The urachus is the embryologic remnant of allantois and the adjoining ventral cloaca. It is a tubular structure and its lumen becomes obliterated with the advancing age. However, in a small proportion of adults (2%), its patency with the urinary bladder persists. [1] In such instances, urachus runs downward in the extraperitoneal space from the umbilicus to the apex of the bladder. Histologically, urachal remnants consist of tubular structures and canals, usually lined the by transitional type epithelium. The lumen of the lower part of the persistent urachus may communicate with the cavity of the bladder. Persistent urachal remnants are more likely a normal phenomenon and become clinically important when complicated by infection, cystic dilation, or when neoplasia develops in them. [2],[3],[4],[5] Focal mucinous glandular metaplasia is often seen and may provide a morphological basis for the development of intestinal type tumors. [2]

Primary urachal adenocarcinoma is a rare and a devastating disease, representing 0.17-0.34% of all bladder tumors [6] and theoretically might occur at any point up to the umbilicus. Carcinomas arising in the urachus or urachal remnants are generally adenocarcinomas, although occasionally, they can have squamous and transitional cell features. Urachal adenocarcinomas are mostly well-differentiated mucinous adenocarcinomas. The next most frequent pattern is the enteric type; these tumors closely resemble colonic adenocarcinomas and must be distinguished from colonic adenocarcinomas involving the bladder. Rarely, urachal adenocarcinomas can have a signet ring pattern. [7],[8],[9]

Immunohistochemically, these tumors are positive for CK20 and can show variable positivity for CK7 and 34BE12. [10] Immunostains do not unequivocally discriminate a urachal from a colorectal carcinoma, but diffuse positivity for 34BE12 would support, and diffuse nuclear immunoreactivity for β-catenin would militate against, a diagnosis of urachal carcinoma. [10]

These relatively rare genitourinary neoplasms usually present as a supravesical mass. Most cases occur in individuals in their 40s and 50s, with a mean age of occurrence approximately 10 years younger than that for carcinoma arising in the urothelium. The identification of adenocarcinomas of urachal origin is based on several clinical and pathologic features, the most important of which are location of the tumor in the bladder dome, location centered in the muscular wall rather than in the mucosa, and the absence of intestinal metaplasia or CIS on the urothelium surface. Also helpful is the identification of urachal remnants, a sharp demarcation between tumor and normal urothelium, and the exclusion of metastatic adenocarcinoma from other sites.

The pathologic stage is an important prognostic factor in urachal carcinoma. Urothelial surface involvement by the carcinoma and the presence of cystitis cystica/glandularis do not necessarily exclude the diagnosis of urachal carcinomas. Urachal carcinomas commonly occur in the bladder dome and are generally treated by partial cystectomy with an en bloc resection of the median umbilical ligament and umbilicus. Local recurrence may be seen owing to seeding within the distal urothelial tract, particularly in tumors with a polypoid configuration that open into the bladder cavity. Prognosis does not appear to be significantly influenced by histology and grade of the tumor. Metastatic disease has a poor response to chemotherapy. Some tumors may respond to cisplatin-based regimens. The overall survival for all stages is 62 months with 34% of the patients showing survival after 5 years. The type of surgery performed may have an effect on local recurrence despite negative margins of resection. [10]

To conclude, misdiagnosis of urachal carcinoma is still a reality. Accurate diagnosis necessitates a high degree of clinical suspicion and imaging studies. Appropriate local treatment is the key for improved survival.

 
 > References Top

1.Hammond G, Yglesias l, Devis J. The Urachus: The anatomy,histology and development. Anat Rec 1941;64:80:271-4.  Back to cited text no. 1
    
2.Schubert GE, Pavkovic MB, Bethke-Bedürftig BA. Tubular urachal remnants in adult bladders. J Urol 1982;127:40-2.  Back to cited text no. 2
    
3.IrwinPP, Weston PM, Sheridan W, Matthews PN. Transitional cell carcinoma arising in a urachal cyst. Br J Urol 1991;61:103-4.  Back to cited text no. 3
    
4.NegroF, Crucitti A. Urachal adenocarcinoma: A case report. Minerva Chir 1994;49:885-8.  Back to cited text no. 4
    
5.Hull MT, Warfel KA. Urachal cystadenoma with abundant glycogen: Ultrastructural study. Ultrastruct Pathol 1994;18:499-502.  Back to cited text no. 5
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6.Fanning DM, Sabah M, Mellotte GJ, Donovan MG, Little DM. An unusual case of cancer of the urachal remnant following repair of bladder exstrophy. Iri J of Med Sci 2009;23:476-9.  Back to cited text no. 6
    
7.Ohira S, Shiohara S, Itoh K, Ashida T, Fukushima M, Konishi I. Urachal adenocarcinoma metastatic to the ovaries: Case report and literature review. Int J Gynecol Pathol 2003;22:189-93.  Back to cited text no. 7
[PUBMED]  [FULLTEXT]  
8.Young RH. Urachal adenocarcinoma metastatic to the ovary simulating primary mucinous cystadenocarcinoma of the ovary: Report of a case. Virchows Arch 1995;426:529-32.  Back to cited text no. 8
[PUBMED]    
9.Trastour C, Desprez B, Delotte J. Ovarian metastases from an urachal adenocarcinoma. Eur J Obstet Gynecol Reprod Biol 2006;125:143-4.  Back to cited text no. 9
    
10.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. 10
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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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