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  Table of Contents    
CASE REPORT  
Year : 2018  |  Volume : 61  |  Issue : 1  |  Page : 127-130
A rare case of primary female urethral adenocarcinoma: Columnar type with colonic adenocarcinoma features


Department of Pathology, A. J. Institute of Medical Sciences and Research Centre, Mangalore, Karnataka, India

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Date of Web Publication22-Mar-2018
 

   Abstract 


Female urethral carcinoma is extremely rare and accounts for 0.02% of all women's cancers and <1% of cancers in the female genitourinary tract. Adenocarcinoma accounts for only 10% of urethral carcinomas in females. Due to their location, presentation is usually late and tumors are often missed on physical examination. As in this case, nonspecific symptoms in the early stages may delay the diagnosis in most patients. Herein, we present an extremely rare case of the columnar type of primary female urethral adenocarcinoma exhibiting colonic adenocarcinoma features which to the authors' best knowledge has not been reported to date. The present study emphasizes the importance of a careful clinical examination and also highlights the role of imaging studies, and biopsy in making an accurate preoperative diagnosis of this rare disease. The disease may have devastating sequelae due to local and metastatic involvement if not recognized and treated earlier.

Keywords: Columnar type, Female Urethral adenocarcinoma, Skene's gland

How to cite this article:
Gangadhar P, Pai MR, Sandhya I. A rare case of primary female urethral adenocarcinoma: Columnar type with colonic adenocarcinoma features. Indian J Pathol Microbiol 2018;61:127-30

How to cite this URL:
Gangadhar P, Pai MR, Sandhya I. A rare case of primary female urethral adenocarcinoma: Columnar type with colonic adenocarcinoma features. Indian J Pathol Microbiol [serial online] 2018 [cited 2019 Dec 10];61:127-30. Available from: http://www.ijpmonline.org/text.asp?2018/61/1/127/228193





   Introduction Top


Female urethral carcinoma is extremely rare and accounts for 0.02% of all women's cancers and <1% of cancers in the female genitourinary tract. Squamous cell carcinoma is the most common histological type of urethral carcinoma in females, accounting for 70% of all cases followed by transitional cell carcinoma (20%) and adenocarcinoma (10%) cell types.[1] Due to their location, presentation is usually late, and tumors are often missed on physical examination, resulting in diagnosis at an advanced stage and a poor outcome.[2]

A search of PubMed literature of primary female urethral columnar type of adenocarcinoma did not reveal any case reports. Herein, we present an extremely rare case of the columnar type of the primary female urethral adenocarcinoma exhibiting colonic adenocarcinoma features which to the authors' best knowledge has not been reported to date. The present study emphasizes the importance of a careful clinical examination and highlights the role of imaging studies and biopsy in making an accurate preoperative diagnosis of this rare disease.


   Case Report Top


A 60-year-old female patient presented with the complaints of increased frequency of micturition and incomplete voiding for one month. Her systemic examination and hematological parameters were within the normal limits. Initial evaluation by ultrasonography and computed tomography (CT) of abdomen/pelvis revealed bilateral hydroureteronephrosis and micturating cystourethrogram demonstrated Grade IV vesicoureteric reflux. [Figure 1] (a) She was initially treated conservatively; in view of persistent symptoms, she underwent detailed evaluation. Per vaginal examination revealed suspicious induration of the urethra. She was further evaluated by using transvaginal ultrasonography [Figure 1] (b) and magnetic resonance imaging (MRI) which revealed an ill-defined hypoechoic lesion in the periurethral region/anterior wall of the vagina with mild internal vascularity on Doppler [Figure 1] (c). Diagnostic cystourethroscopy revealed erosion of the urethra by an ill-defined mass, multiple cold cup biopsies were taken for histopathological examination.
Figure 1: (a) Micturating cystourethrogram showing right sided Grade IV vescico ureteric reflux (arrow) and multiple sacculations in bladder (small arrow). (b) Transvaginal sonography shows an ill-defined hypoechoeic lesion in the periurethral region/anterior wall of vagina (arrow) with mild internal vascularity on Doppler. (c) Magnetic resonance imaging of abdomen and pelvis (plain and contrast) shows an ill-defined lesion in the anterior wall of vagina (arrow) with thickened proximal urethra anterior to it

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Microscopy revealed fragments of malignant tissue composed of papillae, glands, and clusters of malignant cells lined by columnar cells with elongated hyperchromatic pseudostratified nuclei with an eosinophilic to clear cytoplasm [Figure 2] (a). The clusters of glands exhibited mucin (PAS positivity) in the cytoplasm [Figure 2](b), [Figure 2](c). Stroma was extensively infiltrated by mixed inflammatory cells and focal necrosis. The tumor tissue has infiltrated into the muscular wall [Figure 2] (d). Histopathological features were those of adenocarcinoma of the urethra with colonic adenocarcinoma features. Hence, metastatic lesions originating from the intestinal tract/ovaries were ruled out by relevant clinical history, physical examination, colonoscopic examination, and imaging studies. A management plan was formulated in consultation with a tumor board, and the patient was advised to undergo multimodality treatment with chemoradiation. Currently, the patient has completed six cycles of chemotherapy and radiotherapy.
Figure 2: (a) Photomicrograph, shows malignant tissue composed of papillae, glands, and clusters of malignant cells lined by columnar cells having elongated hyperchromatic, psuedostratified nuclei with eosinophilic to clear cytoplasm (arrow) (H and E, ×200). (b and c) The clusters of gland exhibit mucin in the cytoplasm (arrow) which are PAS positive (inset) (H and E, ×400). Luminal necrotic debris also noted (arrow in b). (d) Tumor tissue has infiltrated into the muscular wall (H and E, ×400)

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   Discussion Top


Primary urethral carcinoma in women is rare, being nearly three times more common in males [1] accounting for 0.002% of malignant neoplasms of the female genitourinary tract.[3] The age of presentation ranged from 45 to 72 years with a mean age of 58 years.[2]

There is a striking anatomical distribution of these tumors based on their histology: distal urethral carcinomas are predominantly squamous cell type whereas proximal urethral carcinomas are more often urothelial and adenocarcinoma type.[4]

The two primary histologic subtypes of adenocarcinoma of the urethra are as follows: Columnar/mucinous (“intestinal”) and clear cell type (CCA). Columnar-type adenocarcinoma is most often composed of colonic-type glandular epithelium as in the present case.[3] Primary urethral adenocarcinoma can resemble adenocarcinoma at other sites such as the colon or pancreas on hematoxylin and eosin stained sections.[3] The present case had features of colonic adenocarcinoma.

The origin of urethral adenocarcinomas remains unclear. Certain authors have suggested that urethral adenocarcinomas in females originate at the periurethral Skene's glands, which is a homolog of the prostate. PSA positivity may be considered as evidence in support of this theory of an origin from the Skene's glands.[5] However, larger series and a literature review suggest that only a minority of cases account for PSA reactive tumors. A study by Dodson et al.[6] found that neither the clear cell type nor the columnar/mucinous type stained for PSA. The fact that the majority do not express PSA suggests that urethral adenocarcinoma may be derived from paraurethral glands other than Skene's glands or from tissue other than the paraurethral glands, or it somehow loses the ability to express PSA or that distinct subsets of paraurethral ducts exist only some of which are homologous to the prostate. Therefore, the histogenesis of the majority of these tumors remains unknown.[5] The present case was PSA negative.

Kato et al.[7] used carcinoembryonic antigen (CEA) along with PSA immunohistochemistry and speculated that columnar/mucinous type of adenocarcinoma develops from the proximal part of the paraurethral duct near the urethra. It is positive for CEA whereas the distal part of the duct is rudimentary in structure showed positive staining for PSA.

A differential diagnosis of the primary versus metastatic carcinoma involving the urethra is frequently difficult on histologic grounds alone. However, the clinical sequence of events, distribution of disease, and imaging modalities generally aid in these cases.[8]

There are many etiological factors associated with the development of urethral carcinoma in females some of which are leukoplakia, chronic irritation, caruncles, polyps, parturition, and human papillomavirus infection or other viral infections.[8] Female urethral diverticula may also predispose to malignant change, the most common type being adenocarcinoma.[1]

Most patients with urethral carcinoma present with obstructive symptoms, dysuria, urethral bleeding, urinary frequency, and often a palpable urethral mass or induration as in this case. A suspicion of a urethral tumor should be raised in any otherwise healthy middle-aged female who presents with urinary retention without prior urological history.[8]

Tumors spread generally by local extension and may ulcerate to the skin and vulvar region as the tumor progresses. Proximal lesions may extend posteriorly into the vagina or proximally into the bladder. Lymphatic spread is uncommon in the early stages, but clinically, palpable nodes may be present in up to one-third of patients at presentation and half of patients with advanced and proximal tumors. Hematogenous spread may occur in the lung, liver, bone, and brain, in order of frequency.[1] In the present case, tumor was localized to the distal urethra. There was no lymphadenopathy/metastasis.

The evaluation of females with suspected urethral carcinoma includes cystourethroscopy, physical examination under anesthesia, CT of the abdomen and pelvis and chest radiography. MRI has been used to evaluate pelvic lesions and is beneficial in the determination of local extension. MRI has been reported to be accurate in local staging of urethral tumors in 90% of patients. Transvaginal ultrasonography may also provide clues for diagnosis. Biopsy of a suspected tumor is mandatory to confirm the diagnosis. To establish the diagnosis of primary urethral mucinous adenocarcinoma, exclusion of metastatic lesion originating from the intestinal tract or ovaries is essential.[1]

Due to the rarity of this type of tumor and heterogeneity of the disease, most studies report similar prognoses in different histological subtypes. The prognosis is determined largely by the clinical stage and the location of the lesions. Tumors in the distal urethra tend to have a better outcome.[1]


   Conclusion Top


Due to its rarity, a high index of suspicion of a urethral tumor should be maintained in an otherwise healthy middle-aged female without prior urological history who presents with urinary retention. As in this case, nonspecific symptoms during the early stages may delay the diagnosis in most patients. A careful clinical examination may alert the wary clinician who can then order appropriate imaging studies which are helpful in establishing the diagnosis. Newer generation MRI is generally considered to be superior to CT for local disease staging due to the exquisite soft-tissue resolution without the risk of ionizing radiation. A biopsy of any suspicious urethral lesions is necessary for confirmation. Proper communication between the treating clinician and pathologist is critical before the diagnosis of a primary tumor is established. The disease may have devastating sequelae due to local and metastatic involvement if not recognized and treated earlier. Advanced disease tends to carry a grim prognosis.

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.
Wang X, Bai P, Su H, Luo G, Zhong Z, Zhao X, et al. Management of primary adenocarcinoma of the female urethra: Report of two cases and review of the literature. Oncol Lett 2012;4:951-4.  Back to cited text no. 1
    
2.
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. 2
    
3.
Satyanarayan A, Redd L, Dyer A, Wright A, Walker J. Adenocarcinoma of the urethra with mucinous features. Rev Urol 2015;17:38-41.  Back to cited text no. 3
    
4.
Mehra R, Vats P, Kalyana-Sundaram S, Udager AM, Roh M, Alva A, et al. Primary urethral clear-cell adenocarcinoma: Comprehensive analysis by surgical pathology, cytopathology, and next-generation sequencing. Am J Pathol 2014;184:584-91.  Back to cited text no. 4
    
5.
Murphy DP, Pantuck AJ, Amenta PS, Das KM, Cummings KB, Keeney GL, et al. Female urethral adenocarcinoma: Immunohistochemical evidence of more than 1 tissue of origin. J Urol 1999;161:1881-4.  Back to cited text no. 5
    
6.
Dodson MK, Cliby WA, Pettavel PP, Keeney GL, Podratz KC. Female urethral adenocarcinoma: Evidence for more than one tissue of origin? Gynecol Oncol 1995;59:352-7.  Back to cited text no. 6
    
7.
Kato H, Kobayashi S, Islam AM, Nishizawa O. Female para-urethral adenocarcinoma: Histological and immunohistochemical study. Int J Urol 2005;12:117-9.  Back to cited text no. 7
    
8.
Sharp DS, Angermeier KW. Surgery of penile and urethral carcinoma. In: Wein AJ, editor. Campbell-Walsh Urology. 10th ed. Philadelphia: Elsevier-Saunders; 2011. p. 951-4.  Back to cited text no. 8
    

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Correspondence Address:
Prithal Gangadhar
Department of Pathology, A. J. Institute of Medical Sciences and Research Centre, Mangalore - 575 004, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_745_16

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