A Long-Term Course of a Primary Urethral/Paraurethral Adenocarcinoma

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Int Urogynecol J (2000) 11:392±394 ß 2000 Springer-Verlag London Limited

International Urogynecology Journal

Case Report A Long-Term Course of a Primary Urethral/Paraurethral Adenocarcinoma M. M. Terzic1 and B. V. Stimec2 1

Department of Obstetrics and Gynecology and 2Institute for Anatomy, School of Medicine, Belgrade University, Belgrade, Yugoslavia

Abstract: The authors report a rare case of primary urethral/paraurethral adenocarcinoma in a female patient. The tumor was ®rst detected at the external and internal urethral ori®ces, with later recurrences in the region of the urinary bladder neck. Histology did not reveal the exact origin of the malignancy. The patient was treated by transurethral resection, regularly repeated during the 3±6-monthly checks. Five years after diagnosis she remains symptom free and has a high quality of life. Keywords: Adenocarcinoma; Transurethral resection; Urethral/paraurethral

Introduction Primary carcinoma of the female urethra is a rare neoplasm with a relatively poor prognosis, except when the disease is limited to the anterior or distal urethra. The incidence is less than 0.1% of all female genital malignancies. The majority of urethral lesions are squamous growths originating from the mucosa and, less commonly, adenocarcinomas originating from the paraurethral ducts. Skene's (periurethral) gland carcinoma is a rare neoplasm accounting for less than 0.003% of all genital tract malignancies in females [1]. Correspondence and offprint requests to: Dr Milan M. Terzic, Department of Obstetrics and Gynecology, School of Medicine, Belgrade University, Visegradska 26, 11000 Belgrade, Yugoslavia. Tel: +381 11 361-5592; Fax: +381 11 361-5603; e-mail: [email protected]

We present a case of urethral/paraurethral adenocarcinoma of uncertain origin in a female patient, with an uneventful course of 5 years.

Case Report In September 1994 a 64-year-old woman was admitted to the Department of Gynecology and Obstetrics complaining of frequent micturition, dysuria and hematuria. The onset of symptoms was 9 months prior to admission and was interpreted by a general practitioner as a recurrent urinary tract infection, which had been treated according to the antibiogram. On admission the gynecological examination revealed a 50 6 45 6 30 mm widely pedunculated tumor arising close to the external urethral ori®ce. The tumor had a dilapidated `cauli¯ower' appearance and bled profoundly when touched. The peduncle was tied off completely using two sutures. Tightening of the sutures led to incomplete division of the peduncle, followed by excessive bleeding, which was controlled by a third suture applied to the base of the peduncle. At this point appropriate specimens for biopsy were taken. Within 1 week the tumor necrotized, leaving no remnants. A complete histological work-up of the biopsy samples was performed, including the following strainings of para®n-embedded tissue: hematoxylin±eosin, periodic acid±Schiff (PAS), Giemsa, Alcian blue (AB), immunohistochemistry for prostate-speci®c antigen (PSA), and reticulin staining for basement membrane. The histopathological examination revealed small irregular glandular and elongated papillary formations lined by a single/two-layered columnar epithelium (Fig. 1). The cells had moderately abundant, light basophil, PAS-

Primary Urethral Adenocarcinoma



Fig. 1. Hematoxylin±eosin staining of paraf®n-embedded biopsy section (magni®cation 3206) (see text for descripton).

negative, AB sporadically positive cytoplasm, with alcianophil material in some of the lumina. The nuclei were large, irregularly oval, with small nucleoli and very rare mitoses. The loose connective stroma contained a small number of mononuclear cells and granulocytes. No invasion of blood or lymph vessels was detected. The reaction to PSA was negative. The histopathological diagnosis was well differentiated papillary adenocarcinoma with mild/moderate nuclear atypia, without blood/ lymph vessel invasion. Morphologically the tumor presented as being neither of intestinal nor of urinary bladder origin. The negative PSA reaction could not exclude carcinoma of the Skene's glands. Besides the pathological team from the Department of Obstetrics and Gynecology, the biopsy specimen was carefully and independently examined by two additional groups of pathologists from separate medical institutions. Their ®ndings did not differ from ours in any detail. The patient was referred to the Urology Department, where urethrocystoscopy was performed, revealing urethral papillary protrusions in the region of the bladder neck. These formations were electroresected and underwent histopathological examination. The ®ndings presented the same type of carcinoma as already described. Ultrasound and CT scanning found no tumor or enlarged lymph nodes in the pelvis and abdomen. After the procedure the patient's symptoms decreased signi®cantly, her recovery was uneventful and she was discharged from the department. Six months later she was readmitted for dysuria. The control urethrocystoscopy diagnosed a recurrence of the same papillary tumor in the region of the internal urethral ori®ce. The patient was advised to undergo radical surgery but she refused. A satisfactory transurethral resection (TUR) of the tumor was performed and the histological diagnosis recon®rmed. From that time until the present (5 years) the patient has had a regular 3±6-month urethrocystoscopic check-up, with subsequent TUR of recurrent tumor at the same site. She is feeling well and has a good quality of life.

Generally, adenocarcinomas of the female urethra are assumed to arise from the periurethral glands, the female equivalent of the prostate. Observations by Spencer et al. [2] suggest that clear cell adenocarcinomas arise from the female paraurethral ducts, rather than embryonic remnants. These ducts appear to be homologous to the prostate and in some cases may be misinterpreted as urethral diverticula. Skene's glands are the equivalent of the prostate in females and tumors arising from them are immunohistochemically similar to male prostate carcinoma [3]. On the other hand, a recent study showed that primary adenocarcinoma of the female urethra has multiple tissue origins. Immunohistochemical studies have shown that these tumors arise from glandular metaplasia analogous to the potential histogenesis already demonstrated in the bladder. PSA-positive reactivity could suggest an origin from Skene's glands. Clear cell adenocarcinoma is a third possible pathway in the development of this rare subset of adenocarcinomas [4]. After removal, paraurethral peduncular tumors must be evaluated for their origin: primary or metastatic. In the differential diagnosis of metastatic carcinoma histochemistry can contribute ®rst to the differential diagnosis between carcinoma and other neoplasms, especially sarcoma, and secondly to determination of the type of differentiation within a carcinoma and, if possible, the origin of the neoplasm. In our case we have achieved all of these except precise determination of tumor origin. The controversy was based on a negative PSA reactivity, which could not con®rm nor discard the diagnosis of Skene's gland tumor. In distinguishing between anaplastic carcinoma and sarcoma or lymphoma the arrangement of the tumor cells can be useful. With few exceptions carcinoma cells tend to grow in solid nests and cords surrounded by connective tissue stroma, whereas sarcomas and lymphomas tend to grow diffusely. Reticulin and Giemsa stains will reveal the pattern of reticulin and collagen ®bers, and thereby accentuate the growth pattern of the neoplastic cells, as shown in our examination. Acid phosphatase can be found in a wide variety of tumors. Extremely high activity of this enzyme in an adenocarcinoma, however, is almost diagnostic of prostatic origin. Ebisuno et al. [5] described a case of clear cell adenocarcinoma arising from the female urethra. Histologically, solid and glandular areas consisted of clear cells. The tumor cells stained positively with antibodies to prostate-speci®c antigen and prostatic acid phosphatase, suggesting that the clear cell adenocarcinomas arise from the female paraurethral duct, rather than embryonic remnants. Urethral adenocarcinoma is a rare malignancy whose origin remains controversial. Although the tumor in our case was histologically proved malignant, and despite the lack of radical surgery, the course of the disease has remained uneventful and the patient's quality of life very good. All suspected urethral and periurethral protrustions


should be biopsied prior to treatment in order to rule out urethral adenocarcinoma.

References 1. Dodson MK, Cliby WA, Keeney GL, Peterson MF, Podratz KC. Skene's gland adenocarcinoma with increased serum level of prostate-speci®c antigen. Gynecol Oncol 1994;55:304±307 2. Spencer JR, Brodin AG, Ignatoff JM. Clear cell adenocarcinoma of the urethra: evidence for origin within paraurethral ducts. J Urol 1990;143:122±125

M. M. Terzic and B. V. Stimec 3. Zaviacic M, Sidlo J, Borovsky M. Prostate speci®c antigen and prostate speci®c acid phosphatase in adenocarcinoma of Skene's paraurethral glands and ducts. Virchows Arch A Pathol Anat Histopathol 1993;423:503±505 4. Murphy DP, Pantuck AJ, Amenta PS et al. Female urethral adenocarcinoma: immunohistochemical evidence of more than 1 tissue of origin. J Urol 1999;161:1881±1884 5. Ebisuno S, Miyai M, Nagareda T. Clear cell adenocarcinoma of the female urethra showing positive staining with antibodies to prostate-speci®c antigen and prostatic acid phosphatase. Urology 1995;45:682±685

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