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CASE REPORT  
Year : 2012  |  Volume : 55  |  Issue : 2  |  Page : 245-247
Clear cell adenocarcinoma of the male urethral tract


Department of Pathology, Rajiv Gandhi Cancer Institute and Research Centre, Rohini, Delhi, India

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Date of Web Publication3-Jul-2012
 

   Abstract 

We present a rare case of clear cell adenocarcinoma of the male bulbomembranous urethra. Mostly these tumors have been described in the female urethral tract with its possible origin from mullerian remnants, wolffian remnants or paraurethral glands. Histologically, these tumors have typically tubulocystic pattern comprising of hobnailed cells with clear glycogenated cytoplasm along with well-defined cytoplasmic membranes. This case is being presented due to its rarity, aggressive behavior and to discuss, trauma as its possible etiological factor

Keywords: Bulbomembranous urethra, clear cell adenocarcinoma, prostatic-specific antigen

How to cite this article:
Gandhi JS, Khurana A, Tewari A, Mehta A. Clear cell adenocarcinoma of the male urethral tract. Indian J Pathol Microbiol 2012;55:245-7

How to cite this URL:
Gandhi JS, Khurana A, Tewari A, Mehta A. Clear cell adenocarcinoma of the male urethral tract. Indian J Pathol Microbiol [serial online] 2012 [cited 2020 Feb 25];55:245-7. Available from: http://www.ijpmonline.org/text.asp?2012/55/2/245/97895



   Introduction Top


Clear cell adenocarcinomas (CCA) are rare tumors which have been traditionally described in the female genitourinary tract. According to English literature, previously only six cases have been described in the male urethra. [1] Clinically, these tumors present with obstructive symptoms, hematuria and repeated urinary tract infections (UTI). Histological spectrum can be of tubular, tubulo-papillary or diffuse pattern with typically hobnailed cells with characteristic immunohistochemistry revealing immunopositivity for prostatic-specific antigen (PSA). [2],[3] The strong PSA supports origin of these tumors from paraurethral glands; however, the origin is still unclear due to the paucity of cases. To our knowledge we report the seventh case of CCA of male urethra.


   Case Report Top


A 55-year-old male presented to our institution with history of repeated attacks of urinary tract infection along with urinary obstructive symptoms. He had these symptoms since last 6 years subsequent to a road traffic accident (RTA) in which he suffered perineal tears with injury to the bulbomembranous part of the urethra. A repair and reconstructive surgery was performed, however the patient was symptomatic due to urethral strictures for which he underwent multiple sessions of urethral dilatations using urethral dilatators.

Three months prior to the present complaint, he noticed a perineal nodule which had progressive growth with rapid change in size from a peanut to an apricot. Fine needle aspiration cytology was done from the nodule which revealed cellular smears comprising of neoplastic epithelial cells arranged in small groups and acini with peripheral palisading. The cells showed eccentric nuclei with prominent nucleoli. The background revealed dense neutrophillic inflammation with satellitosis around the epithelial cell clusters. Considering the morphology a possibility of poorly differentiated adenocarcinoma was offered [Figure 1]a. A contrast enhanced magnetic resonance imaging (MRI) scan was performed which revealed a heterogeneously enhancing soft tissue mass involving the membranous, bulbous and penile urethra over a length of 9 cm with diffuse enhancing thickening in the remaining penile urethra upto the meatus [Figure 1]b. Prostate was also enlarged in size measuring 4.6(AP) × 4.7(CC) × 3.6(Tr) cm. Peripheral zones were unremarkable. The urinary bladder showed thick irregular trabeculations with inadequate distension. Bilateral inguinal lymph nodes were enlarged upto subcentimeter size. Due to the enlarged prostate gland, his serum PSA was done which was 0.4 ng/ml.
Figure 1: (a): Romanoswky-stained smear showing neoplastic cells in cohesive fragments as well as dispersed singly. (Giemsa; ×200). (b): MRI showing a diff use thickening of the bulbomembranous and penile urethra (Red Arrow). (c): Urethrectomy specimen showing diffuse thickening
of the bulbomembranous urethra with the growth, obliterating the lumen


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The patient underwent a radical cysto-prostatourethrectomy with bilateral ilioinguinal block dissection with an ileal conduit. The patient had an un-eventful post operative course. On gross examination the prostate was mildly enlarged and measured 5.5 × 4.5 × 3.5 cm. On serial slicing no definite nodule was observed. The uretherectomy specimen measured 14 cm in length with a diameter of 2.7 cm. On serial slicing a grey white circumferential tumor was seen filling 7 cm of length of bulbomembranous urethra with other dimension being 2.5 cm and a depth of 1.8 cm [Figure 1]c. On routine hematoxylin and eosin staining (H & E), the tumor was seen arranged in tubulocystic and tubulopapillary pattern lined by epithelial cells with hobnailed morphology with clear cytoplasm and well-defined cytoplasmic membrane. The cells had high grade nuclear morphology with vesicular nuclei and prominent nucleoli [Figure 2]a and b. The glands were studded in a dense neutrophilic background. The tumor was infiltrating the periurethral muscle and corpus spongiosum (pT2). On examining the prostate, an incidental prostatic acinar adenocarcinoma, Gleason score 3 + 3 = 6 was discovered in the left posterior upper one third of the peripheral region of prostate [Figure 2]c. All the lymph nodes dissected were free.
Figure 2: (a): Section from male bulbomembranousurethra showing neoplastic epithelial cells arranged in tubuloglandular architecture. (Hematoxylin and Eosin; ×100). (b): Section from male bulbomembranous urethra showing high grade neoplastic epithelial cells with hobnailing, vesicular chromatin and prominent nucleoli. (Hematoxlin and Eosin; ×200). (c): Section from prostate showing incidental prostatic acinar adenocarcinoma. (Hematoxylin and Eosin; ×100)

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On immunohistochemistry (IHC) the tumor cells from the male urethra, expressed PSA, PSAP and CA-125 [Figure 3]. Immunostains for p63 and CK20 were not expressed in the tumor cells.
Figure 3: (a): Immunostains with prostate-specific antigen showing cytoplasmic positivity. (DAB; ×100). (b): Immunostains with prostate-specific acid phosphatase showing cytoplasmic positivity. (DAB; ×100). (c): CA125 immunostaining of a clear cell adenocarcinoma revealing strong immunopositivity. (DAB; ×100)

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Hence a final histopathological diagnosis of CCA of bul-bomembranous urethra (pT2) along with acinar adenocarcinoma of prostate (pT2a) was offered.


   Discussion Top


CCA of the male urethra is an extremely rare tumor with only six documented cases in the world English literature. Previously these tumors were known as mesonephric adenocarcinomas due to its supposed mesonephric origin. The term CCA was proposed by Young and Scully in 1985 due to its histopathological spectrum similar to CCA of female genital tract. Various hypothesis have been postulated for its origin, which include (1) Mullerian origin (2) Glandular differentiation of urothelium (3) vesicular adenocarcinoma of non mullerian origin. [2] Histologically, CCAs have typical tubulopapillary architecture lined by hobnailed cells with high grade nuclear and cytoplasmic features. [4] These cases can exhibit PSA, PSAP, CA125, CK7 and CK20 immunopositivity. Our case exhibited CA125, PSA and PSAP immunopositivity which supports to some extent the origin of these tumors from Cowper's (bulbourethral) gland. Glandular differentiation of urothelial carcinoma was ruled out by the fact that the IHC studies were negative for p-63 and CK20 immunostains.

Usual clinical presentation of these neoplasms are of obstructive symptoms and hematuria. [1] Recently, Varachhia et al., [5] documented a case of urethral CCA presenting as periurethral abscess and fistulae. None of the previously diagnosed cases had a history of trauma as compared to the indexed case which was associated with RTA and injury to bulbomembranous part of urethra. Due to the limited number of similar reported cases, injury to the urethra as a possible etiological cause of developing a CCA is only a supposition. More cases needs to be evaluated clinically before considering trauma as a possible etiological factor.

Early surgical intervention is definitely a good treatment option. Cantrell et al., [6] treated their male patient with total pelvic radiation. Spencer et al., [7] on reviewing the female cases of CCA, postulated that it was probably best to proceed with exenteration in patients who presented with resectable disease as compared to radiotherapy alone. In a case report by Gogus et al., [1] their patient was treated with radical cystoprostatectomy including bilateral inguinal and pelvic lymph node dissection and urethrectomy with ileal conduit urinary diversion along with three cycles of methotrexate-vinblastine-epirubicin-cisplatin chemotherapy. Ziedman et al., [8] have proposed en bloc resection of penis, urethra, scrotum and anterior pubis with cystoprostatectomy. The optimal treatment in males still needs to be formulated owing to the paucity of the described entity. Our patient underwent cystoprostatourethrectomy with bilateral ilioinguinal block dissection with an ileal conduit. He is further planned for cisplatin-based chemotherapy.


   Conclusions Top


CCA of the male urethra is an extremely rare entity with only six cases in the world English literature having an extremely poor prognosis and uncertain histogenesis. This case is being presented firstly due to its rarity in male gender, secondly due its possible (? etiological) association with traumatic injury, and further more on its incidental association with prostatic acinar adenocarcinoma.

 
   References Top

1.Gogus C, Baltaci S, Orhan D, Yaman O. Clear cell adenocarcinoma of the male urethra. J Urol 2003;10:348-9.  Back to cited text no. 1
    
2.Sun K, Huan Y, Unger PD. Clear cell adenocarcinoma of urinary bladder and urethra: Another urinary tract lesion immunoreactive for P504S. Arch Pathol Lab Med 2008;132:1417-22.  Back to cited text no. 2
[PUBMED]  [FULLTEXT]  
3.Kawano K, Yano M, Kitahara S, Yasuda K. Clear cell adenocarcinoma of the female urethra showing strong immunostaining for prostate-specific antigen. BJU Int 2001;87:412-3.  Back to cited text no. 3
[PUBMED]  [FULLTEXT]  
4.Trabelsi A, Abdelkrim SB, Rammeh S, Stita W, Sorba NB, Mokni M, et al. Clear cell adenocarcinoma of a female urethra: A case report and review of the literature. North Am J Med Si 2009;1:321-3.  Back to cited text no. 4
    
5.Varachhia SA, Goetz L, Persad R, Naraynsingh V. Clear cell carcinoma of the male urethra presenting as periurethral abscess with fistulae. J Pelvic Med Surg 2009;15:221-3.  Back to cited text no. 5
    
6.Cantrell BB, Leifer G, Deklerk DP, Eggleston JC. Papillary adenocarcinoma of the prostatic urethra with clear-cell appearance. Cancer 1981;48:2661-7.  Back to cited text no. 6
[PUBMED]    
7.Spencer JR, Brodin AR, Ignatoff JM. Clear cell adenocarcinoma of the urethra: Evidence for origin within paraurethral ducts. J Urol 1990;143:122-5.  Back to cited text no. 7
    
8.Zeidman EJ, Desmond P, Thompson IM. Surgical treatment of carcinoma of the male urethra. Urol Clin North Am 1992;19:359-72.  Back to cited text no. 8
[PUBMED]    

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Correspondence Address:
Jatin S Gandhi
C-2-C/12/58, Janak Puri, New Delhi - 110 058
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.97895

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    Figures

  [Figure 1], [Figure 2], [Figure 3]

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