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CORRESPONDENCE
Year : 2018  |  Volume : 14  |  Issue : 2  |  Page : 468-470

Clear cell adenocarcinoma of the urethra with inguinal lymph node metastases: A rare case report and review of literature


1 Department of Pathology, Dr. B. R. Ambedkar Medical College, Bengaluru, Karnataka, India
2 Department of Urology, Institute of Nephrourology, Bengaluru, Karnataka, India

Date of Web Publication8-Mar-2018

Correspondence Address:
Dr. Varuna Mallya
Department of Pathology, Dr. B. R. Ambedkar Medical College, Bengaluru
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.226734

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 > Abstract 


Urethral cancer is a rare malignancy with urothelial subtype being the most common followed by adenocarcinoma. In women, the usual presentation is urinary retention and hematuria. Clear cell variety of adenocarcinoma is a rarer entity which usually has a better prognosis than other variants. Lymph node metastasis is seen in about 30% of urethral cancers. Histopathological differential diagnosis includes Skene gland carcinoma and nephrogenic adenoma. Herein, we present a 58-year-old female patient who presented for evaluation of lymph node mass in the inguinal region. The detailed evaluation revealed clear cell adenocarcinoma of the urethra as the primary with lymph node metastasis.

Keywords: Clear cell adenocarcinoma, nephrogenic adenoma, Skene gland carcinoma, urethral carcinoma


How to cite this article:
Mallya V, Mallya A, Gayathri J. Clear cell adenocarcinoma of the urethra with inguinal lymph node metastases: A rare case report and review of literature. J Can Res Ther 2018;14:468-70

How to cite this URL:
Mallya V, Mallya A, Gayathri J. Clear cell adenocarcinoma of the urethra with inguinal lymph node metastases: A rare case report and review of literature. J Can Res Ther [serial online] 2018 [cited 2019 Nov 14];14:468-70. Available from: http://www.cancerjournal.net/text.asp?2018/14/2/468/226734




 > Introduction Top


Urethral carcinoma constitutes <1% of all the malignancies affecting the female genitourinary system.[1],[2] Historically thought to be more common in women, recent analysis of surveillance, epidemiology, and end results database from the USA during 1973 to 2002 has revealed higher numbers in men than in women.[3] Among women the most common subtype is urothelial followed by adenocarcinoma and squamous cell carcinoma.[3] Mostly seen in elderly women, it presents with urinary obstruction, urethral bleeding, frequency, and dysuria. In women, since the urethra is short,[2],[3] it can get totally involved. Inguinal lymph node metastasis is seen in only 30% of the cases.[1],[2] Clear cell adenocarcinoma of the urethra, a rare histological subtype has good prognosis and is stated to have a mesonephric differentiation.[4] We present a case of a 58-year-old woman who presented to our hospital with swelling in the inguinal region and mild lower urinary tract symptoms (LUTSs). She was subsequently diagnosed with clear cell adenocarcinoma of the urethra.


 > Case Report Top


A 58-year-old woman presented with complaints of a solitary left sided inguinal swelling. History revealed mild LUTS with occasional hematuria, dysuria, and increased frequency. She also gave a history of being treated for recurrent urinary tract infections (UTIs) but had negative urinary culture during each episode. Urine routine microscopy revealed numerous erythrocytes and 10–12 pus cells. Inguinal node mass was firm in consistency and immobile measuring 4 cm × 4 cm. Fine needle aspiration cytology (FNAC) was done, and the smears obtained were cellular with pleomorphic cells having increased nuclear: Cytoplasm ratio and clear cytoplasm. Tendency to acini formation was also seen in [Figure 1]. A diagnosis of metastatic clear cell adenocarcinoma was made. Computed tomography (CT) scan of the abdomen with pelvis revealed circumferential thickening of the urethra and bladder, neck, and base region [Figure 2]. No lymph node enlargement was noticed within the pelvis.
Figure 1: Pleomorphic cells having increased nuclear cytoplasm ratio and clear cytoplasm with tendency to acini formation seen (Giemsa, ×400)

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Figure 2: Computed tomography scan showing circumferential thickening of the urethra and bladder neck and base region

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Urethrocystoscopy was performed which revealed a friable mass in the urethra which bled on touch and occupied almost whole of urethra and bladder neck region. The bladder mucosa was hyperemic and trabeculated. Ureteral orifices were uninvolved. A biopsy was taken from the urethral mass. On histopathology, tumor cells were seen arranged in tubulo cystic, papillary, and diffuse pattern. The individual cells were round to polygonal with clear cytoplasm, large nucleus, coarsely clumped chromatin, and prominent nucleoli [Figure 3]a. Hob nailing was identified [Figure 3]b. A diagnosis of clear cell adenocarcinoma was made. On immunohistochemistry (IHC), the tumor cells were positive for carcinoembryonic antigen (CEA) and negative for prostate-specific antigen (PSA) and prostatic acid phosphatase (PAP).
Figure 3: Photomicrograph showing round to polygonal tumor cells with clear cytoplasm, large nucleus, coarsly clumped chromatin, and prominent nucleoli (H and E, ×400)

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The patient was planned for cystourethrectomy, ileal conduit urinary diversion, and lymph node dissection but has not followed up for the same.


 > Discussion Top


Urethral carcinoma is a very rare tumor. It is seen more commonly in females than males, and the usual age of presentation is sixth to seventh decade.[4],[5] The tumor presents usually with urinary retention. Increased frequency of micturition and dysuria are the other common symptoms.[2] In males, distal urethral involvement presents with early clinical symptoms.[6] Proximal urethral lesions come to notice quite late and tend to metastasize. In women, usually, the entire urethra is involved. Many of the patients give a history of obstructive LUTS and urethral dilatation.[2],[6] Our patient was a 58-year-old lady, who presented with a swelling in the inguinal region. The swelling was present since 2 weeks, was 4 cm × 4 cm, firm in consistency and fixed. It was only subsequently that she also complained of urinary retention although she did give a history of occasional hematuria and dysuria.

Adenocarcinoma makes up 29% of all urethral cancers in women and is said to arise from periurethral glands.[4] It is also known to be associated with urethral diverticula.[2] On FNAC of inguinal lymph node, our patient was diagnosed to have metastatic clear cell adenocarcinoma, and a CT scan of the pelvis revealed circumferential thickening of the urethra and bladder base region. Cystoscopy and a urethral biopsy confirmed a diagnosis of adenocarcinoma of the urethra, clear cell type. Clear cell adenocarcinoma is thought to be of mesonephric origin, konnack used the term “mesonephric carcinoma” and postulated that it arises from mesonephric ducts or intermediate mesodermal vestiges.[7] Clear cell adenocarcinoma is characterized by tumor cells that have clear cytoplasm, enlarged nucleus, and prominent nucleoli arranged in classical triad that is, the tubulo cystic, papillary, and diffuse pattern. Hob nailing is also noted.[2],[4] All these findings were seen in our case. CEA was positive, whereas PSA and PAP were negative.

Following differential diagnosis was considered: Metastasis from female genital tract organs, bladder carcinoma, nephrogenic adenoma, and Skene gland carcinoma. Metastasis from clear cell carcinoma of female internal genitalia was ruled out since no organ involvement was noted on CT scan.[2] Primary bladder carcinoma with the involvement of urethra could be excluded as mass was predominantly in the urethra on cystoscopy. The cells here were very pleomorphic with coarse nuclear chromatin and prominent nucleoli, this easily helped us exclude nephrogenic adenoma, wherein the cells are bland with no pleomorphism. Our case was negative for PSA and PAP on IHC. This helped in affirmation of primary clear cell adenocarcinoma of urethra thereby excluding a Skene gland carcinoma. Skene gland carcinoma arises from the luminal secretary cells of the Skene glands which are the homolog of prostate and just like carcinoma of the prostate are positive for PSA and PAP.[2],[4]

This patient presented atypically with lymph node metastasis and mild LUTS. Urethral cancer should be kept in mind, especially when dealing with elderly females presenting with LUTS or urinary retention and a history of recurrent UTI like features but turn out to be urine culture negative.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 > References Top

1.
Fagan GE, Hertig AT. Carcinoma of the female urethra; review of the literature; report of eight cases. Obstet Gynecol 1955;6:1-11.  Back to cited text no. 1
    
2.
Kurman RJ. Blaustein's Pathology of Female Genital Tract. 5th ed. New York: Springer; 2002. p. 122  Back to cited text no. 2
    
3.
Grivas PD, Davenport M, Montie JE, Kunju LP, Feng F, Weizer AZ. Urethral cancer. Hematol Oncol Clin North Am 2012;26:1291-314.  Back to cited text no. 3
    
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. N Am J Med Sci 2009;1:321-3.  Back to cited text no. 4
    
5.
Oliva E, Young RH. Clear cell adenocarcinoma of the urethra: A clinicopathologic analysis of 19 cases. Mod Pathol 1996;9:513-20.  Back to cited text no. 5
    
6.
Thyavihally YB, Wuntkal R, Bakshi G, Uppin S, Tongaonkar HB. Primary carcinoma of the female urethra: Single center experience of 18 cases. Jpn J Clin Oncol 2005;35:84-7.  Back to cited text no. 6
    
7.
Konnak JW. Mesonephric carcinoma involving the urethra. J Urol 1973;110:76-8.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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