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Undiagnosed urethral carcinoma: an unusual cause of female urinary retention
CASE REPORT
UNDIAGNOSED URETHRAL CARCINOMA: AN UNUSUAL CAUSE OF FEMALE URINARY RETENTION K. C. KOBASHI, T. H. HONG,
AND
G. E. LEACH
ABSTRACT Female urinary retention is extremely rare. Two cases of female urethral carcinoma that presented as urinary retention are reviewed and discussed. UROLOGY 55: 436x–436xiii, 2000. © 2000, Elsevier Science Inc.
T
he incidence of primary urethral carcinoma in females is less than 1%.1 In patients with urinary retention or obstructive voiding symptoms, an increased index of suspicion for urethral carcinoma is imperative in order to provide early aggressive therapy for this malignancy. We report 2 cases of female urethral carcinoma presenting with urinary retention. CASE REPORTS
CASE 1 A 73-year-old woman presented with symptoms of stress urinary incontinence and irritative/obstructive voiding complaints including frequency, decreased force of stream, and a sensation of incomplete bladder emptying. Evaluation revealed microhematuria and a midurethral diverticulum demonstrated on intravenous urogram and voiding cystourethrogram (VCUG) (Fig. 1). Cystoscopy confirmed a midurethral communication site at the 8 o’clock position. Urodynamics studies confirmed stress urinary incontinence with no detrusor instability. The patient underwent an uncomplicated urethral diverticulectomy and bladder neck suspension. The pathology report revealed a urethral diverticulum containing well-differentiated stromal glandular structures thought to represent endometriosis. The patient subsequently experienced recurrent urinary retention despite multiple urethral dilations, a urethrolysis, and an internal urethrotomy. She was referred to us to determine the cause of From Virginia Mason Medical Center, Seattle, Washington Address for correspondence: Kathleen C. Kobashi, M.D., Virginia Mason Medical Center, 1100 9th Avenue, Seattle, WA 98101 Submitted: March 18, 1999, accepted (with revisions): September 20, 1999.
436x
© 2000, ELSEVIER SCIENCE INC. ALL RIGHTS RESERVED
urinary retention. Pelvic examination revealed a hyperelevated and fixed urethra with no other abnormalities. Multichannel water cystometry and pressure-flow analysis demonstrated severe detrusor instability with bladder outlet obstruction and detrusor pressures of up to 100 cm H2O beginning at bladder volumes of 27 cc. Cystoscopy with a 20F female urethroscope showed necrotic tissue on the floor of the urethra. Transurethral biopsy confirmed high-grade, stage IV (T4N0M0) adenocarcinoma. On review of the original pathology slides from the urethral diverticulectomy, the previously described “endometriosis” was thought to represent adenocarcinoma. Computed tomography (CT) scan of the abdomen and pelvis showed a 3-cm mass surrounding the proximal urethra with no evidence of lymphadenopathy or local invasion (Fig. 2). Gastrointestinal and gynecologic evaluation revealed no other primary source for the malignancy. An anterior exenteration with ileal conduit was performed. Pathologic examination showed moderately differentiated urethral adenocarcinoma, extending into the anterior vaginal wall, positive surgical margins, and lymphatic and perineural invasion. Six months postoperatively, the patient received 4500 cGy of external beam radiation therapy to the pelvis. The patient received 5-fluorouracil during radiation therapy to provide a synergistic antineoplastic effect. Approximately 18 months after her pelvic exenteration, the patient underwent resection of recurrent adenocarcinoma in the remaining vaginal wall with labial cutaneous flap reconstruction. Further adjuvant therapy is pending. CASE 2 A 57-year-old woman was referred for evaluation of new-onset urinary retention of unclear etiology. 0090-4295/00/$20.00 PII S0090-4295(99)00446-X
FIGURE 2. Pelvic CT scan shows a mass (arrow) surrounding the proximal urethra with no associated lymphadenopathy.
FIGURE 1. Voiding cystourethrogram reveals a midurethral diverticulum with a filling defect (arrow).
tion, with involvement of the vagina and bladder neck measuring 4.6 ⫻ 4.5 ⫻ 3.4 cm. COMMENT
Before referral, the patient had been evaluated with an extensive work-up including abdominopelvic CT scan and magnetic resonance imaging (MRI) and lumbosacral spine MRI. The abdominopelvic studies revealed a questionable paraurethral mass which was confirmed by pelvic ultrasound. The MRI of the spine was normal. The patient had no significant medical history, no previous pelvic surgery, and no neurologic complaints. Water cystometry and pressure-flow studies revealed a highpressure, low-flow voiding pattern with a postvoid residual volume of 350 cc, consistent with bladder outlet obstruction (Fig. 3). On cystourethroscopy with a 20F female urethroscope, there was difficulty passing the scope into the urethra, deviation of the urethra to the left, and irregular urethral mucosa on the right side of the proximal urethra (Fig. 4). The bladder appeared mildly trabeculated with no obvious extension of the mass into the bladder. Pelvic examination and palpation of the anterior vaginal wall over the scope confirmed a firm mass lateral to the right proximal urethra. The mass was biopsied both transurethrally with cold-cup biopsy forceps and transvaginally with a biopsy needle. Pathologic examination revealed poorly differentiated transitional cell carcinoma (Fig. 5). Metastatic work-up, including chest xray, liver function tests, and the CT scan and MRI mentioned above, was negative. The patient underwent anterior pelvic exenteration with continent urinary diversion. Final pathologic examination revealed a pT3N0M0, grade IV transitional cell carcinoma of the urethra with squamous differentiaUROLOGY 55 (3), 2000
Primary female urethral cancer comprises less than 1% of all malignancies.1 The most common histopathology in a nondiverticular urethral malignancy is squamous cell carcinoma.1,2 The incidence of female urethral diverticula is reported to be 0.6% to 6%,2 although the true incidence is probably higher.3 Fewer than 100 cases of malignancy in female urethral diverticulum have been reported in the world literature,3–5 and only 1 case of urethral diverticular endometriosis has been reported.6 Adenocarcinoma is the most common histopathology detected within female urethral diverticula (61%), with only 12% showing squamous cell carcinoma despite the fact that the majority of the urethra is lined with squamous epithelium.2 VCUG may reveal a diverticulum,3 and filling defects within the diverticulum may represent a stone, blood clot, or malignancy. Factors that should heighten the suspicion of female urethral malignancy include urinary retention of unclear etiology, especially in patients with no neurologic symptoms, no prior pelvic surgery, and no pelvic prolapse. Cystourethroscopy with a 20F female urethroscope with a 0° or 30° lens should be performed, at which time the urethra is palpated over the cystoscopic sheath through the anterior vaginal wall. It is important to use the female scope to provide full visualization of the entire circumference of the urethra, which is not possible with the beaked male sheath. Difficulty passing the scope into the bladder, deviation of the urethra, irregular urethral mucosa, or a palpable firm mass should raise the suspicion of malig436xi
FIGURE 3. Urodynamics illustrating bladder outlet obstruction, with high detrusor pressures (adequate bladder function), no flow despite the patient’s effort to void, and high postvoid residual volume (incomplete emptying).
FIGURE 4. Cystoscopic view of the urethra showing irregular mucosa overlying a right proximal urethral mass.
FIGURE 5. Histopathology revealing poorly differentiated transitional cell carcinoma of the urethra beneath vaginal epithelium.
nancy. Transurethral biopsies with cold-cup biopsy forceps and transvaginal needle biopsies of the urethral mass should be performed promptly to expedite early treatment of this aggressive malignancy.
The treatment for urethral malignancy is wide local excision for localized disease.6 Distal urethrectomy has been performed for distal disease1; however, in women wide local excision typically requires anterior pelvic exenteration. Adjuvant ra-
436xii
UROLOGY 55 (3), 2000
diation therapy or chemotherapy may be administered for extensive disease or for those patients who are not surgical candidates.5,7 However, despite aggressive therapy for urethral carcinoma, recurrence rates are high and the optimal course of therapy has not been determined. CONCLUSIONS Bladder outlet obstruction in women with no neurologic complaints, no history of pelvic surgery, and no pelvic prolapse should raise the suspicion of urethral malignancy. Urethral malignancy must always be included in the differential diagnosis of any female patient with obstructive symptoms. This is especially true when obstruction exists concomitant with a urethral diverticulum, particularly when accompanied by significant firmness on physical examination, a noncalcified filling defect on voiding cystourethrogram, irregular urethral mucosa or a visible lesion emanating from the communication site on cystoscopy, expression of blood or purulence from the diverticulum, or any degree of hematuria. High clinical suspicion and accurate preoperative diagnosis will
UROLOGY 55 (3), 2000
result in the proper aggressive therapy necessary in treatment of this rare disease. ACKNOWLEDGMENT. To Delver R. Cain, M.D., Pathology, for review of the pathologic studies and other contributions. REFERENCES 1. Gheiler EL, Tefilli MV, Tigert R, et al: Management of primary urethral cancer. Urology 52: 487– 493, 1998. 2. Leach GE, and Trockman BA: Surgery for fistulas and diverticulum, in Walsh PC, Retik AB, Vaughn ED, et al (Eds): Campbell’s Urology, 7th ed. Philadelphia, WB Saunders, 1997, pp 1141–1151. 3. Kobashi KC, and Leach GE: Diverticulum and urethral fistula, in Cardozo L, and Staskin D (Eds): Textbook of Female Urology and Urogynecology. Oxford, United Kingdom, Isis Medical Media, 2000. 4. Evans KJ, McCarthy MP, and Sands JP: Adenocarcinoma of a female urethral diverticulum: case report and review of the literature. J Urol 126(1): 124 –126, 1981. 5. Poore RE, and McCullough DL: Urethral carcinoma, in Gillenwater JY, Grayhack JT, Howards SS, et al (Eds): Adult and Pediatric Urology, 3rd ed. Salem, Mosby, pp 1846 –1847. 6. Palagiri A: Urethral diverticulum with endometriosis. Urology 11: 271–272, 1978. 7. Gonzalez MO, Harrison ML, and Boileau MA: Carcinoma in diverticulum of female urethra. Urology 26: 328 – 332, 1985.
436xiii
UNDIAGNOSED URETHRAL CARCINOMA: AN UNUSUAL CAUSE OF FEMALE URINARY RETENTION K. C. KOBASHI, T. H. HONG,
AND
G. E. LEACH
ABSTRACT Female urinary retention is extremely rare. Two cases of female urethral carcinoma that presented as urinary retention are reviewed and discussed. UROLOGY 55: 436x–436xiii, 2000. © 2000, Elsevier Science Inc.
T
he incidence of primary urethral carcinoma in females is less than 1%.1 In patients with urinary retention or obstructive voiding symptoms, an increased index of suspicion for urethral carcinoma is imperative in order to provide early aggressive therapy for this malignancy. We report 2 cases of female urethral carcinoma presenting with urinary retention. CASE REPORTS
CASE 1 A 73-year-old woman presented with symptoms of stress urinary incontinence and irritative/obstructive voiding complaints including frequency, decreased force of stream, and a sensation of incomplete bladder emptying. Evaluation revealed microhematuria and a midurethral diverticulum demonstrated on intravenous urogram and voiding cystourethrogram (VCUG) (Fig. 1). Cystoscopy confirmed a midurethral communication site at the 8 o’clock position. Urodynamics studies confirmed stress urinary incontinence with no detrusor instability. The patient underwent an uncomplicated urethral diverticulectomy and bladder neck suspension. The pathology report revealed a urethral diverticulum containing well-differentiated stromal glandular structures thought to represent endometriosis. The patient subsequently experienced recurrent urinary retention despite multiple urethral dilations, a urethrolysis, and an internal urethrotomy. She was referred to us to determine the cause of From Virginia Mason Medical Center, Seattle, Washington Address for correspondence: Kathleen C. Kobashi, M.D., Virginia Mason Medical Center, 1100 9th Avenue, Seattle, WA 98101 Submitted: March 18, 1999, accepted (with revisions): September 20, 1999.
436x
© 2000, ELSEVIER SCIENCE INC. ALL RIGHTS RESERVED
urinary retention. Pelvic examination revealed a hyperelevated and fixed urethra with no other abnormalities. Multichannel water cystometry and pressure-flow analysis demonstrated severe detrusor instability with bladder outlet obstruction and detrusor pressures of up to 100 cm H2O beginning at bladder volumes of 27 cc. Cystoscopy with a 20F female urethroscope showed necrotic tissue on the floor of the urethra. Transurethral biopsy confirmed high-grade, stage IV (T4N0M0) adenocarcinoma. On review of the original pathology slides from the urethral diverticulectomy, the previously described “endometriosis” was thought to represent adenocarcinoma. Computed tomography (CT) scan of the abdomen and pelvis showed a 3-cm mass surrounding the proximal urethra with no evidence of lymphadenopathy or local invasion (Fig. 2). Gastrointestinal and gynecologic evaluation revealed no other primary source for the malignancy. An anterior exenteration with ileal conduit was performed. Pathologic examination showed moderately differentiated urethral adenocarcinoma, extending into the anterior vaginal wall, positive surgical margins, and lymphatic and perineural invasion. Six months postoperatively, the patient received 4500 cGy of external beam radiation therapy to the pelvis. The patient received 5-fluorouracil during radiation therapy to provide a synergistic antineoplastic effect. Approximately 18 months after her pelvic exenteration, the patient underwent resection of recurrent adenocarcinoma in the remaining vaginal wall with labial cutaneous flap reconstruction. Further adjuvant therapy is pending. CASE 2 A 57-year-old woman was referred for evaluation of new-onset urinary retention of unclear etiology. 0090-4295/00/$20.00 PII S0090-4295(99)00446-X
FIGURE 2. Pelvic CT scan shows a mass (arrow) surrounding the proximal urethra with no associated lymphadenopathy.
FIGURE 1. Voiding cystourethrogram reveals a midurethral diverticulum with a filling defect (arrow).
tion, with involvement of the vagina and bladder neck measuring 4.6 ⫻ 4.5 ⫻ 3.4 cm. COMMENT
Before referral, the patient had been evaluated with an extensive work-up including abdominopelvic CT scan and magnetic resonance imaging (MRI) and lumbosacral spine MRI. The abdominopelvic studies revealed a questionable paraurethral mass which was confirmed by pelvic ultrasound. The MRI of the spine was normal. The patient had no significant medical history, no previous pelvic surgery, and no neurologic complaints. Water cystometry and pressure-flow studies revealed a highpressure, low-flow voiding pattern with a postvoid residual volume of 350 cc, consistent with bladder outlet obstruction (Fig. 3). On cystourethroscopy with a 20F female urethroscope, there was difficulty passing the scope into the urethra, deviation of the urethra to the left, and irregular urethral mucosa on the right side of the proximal urethra (Fig. 4). The bladder appeared mildly trabeculated with no obvious extension of the mass into the bladder. Pelvic examination and palpation of the anterior vaginal wall over the scope confirmed a firm mass lateral to the right proximal urethra. The mass was biopsied both transurethrally with cold-cup biopsy forceps and transvaginally with a biopsy needle. Pathologic examination revealed poorly differentiated transitional cell carcinoma (Fig. 5). Metastatic work-up, including chest xray, liver function tests, and the CT scan and MRI mentioned above, was negative. The patient underwent anterior pelvic exenteration with continent urinary diversion. Final pathologic examination revealed a pT3N0M0, grade IV transitional cell carcinoma of the urethra with squamous differentiaUROLOGY 55 (3), 2000
Primary female urethral cancer comprises less than 1% of all malignancies.1 The most common histopathology in a nondiverticular urethral malignancy is squamous cell carcinoma.1,2 The incidence of female urethral diverticula is reported to be 0.6% to 6%,2 although the true incidence is probably higher.3 Fewer than 100 cases of malignancy in female urethral diverticulum have been reported in the world literature,3–5 and only 1 case of urethral diverticular endometriosis has been reported.6 Adenocarcinoma is the most common histopathology detected within female urethral diverticula (61%), with only 12% showing squamous cell carcinoma despite the fact that the majority of the urethra is lined with squamous epithelium.2 VCUG may reveal a diverticulum,3 and filling defects within the diverticulum may represent a stone, blood clot, or malignancy. Factors that should heighten the suspicion of female urethral malignancy include urinary retention of unclear etiology, especially in patients with no neurologic symptoms, no prior pelvic surgery, and no pelvic prolapse. Cystourethroscopy with a 20F female urethroscope with a 0° or 30° lens should be performed, at which time the urethra is palpated over the cystoscopic sheath through the anterior vaginal wall. It is important to use the female scope to provide full visualization of the entire circumference of the urethra, which is not possible with the beaked male sheath. Difficulty passing the scope into the bladder, deviation of the urethra, irregular urethral mucosa, or a palpable firm mass should raise the suspicion of malig436xi
FIGURE 3. Urodynamics illustrating bladder outlet obstruction, with high detrusor pressures (adequate bladder function), no flow despite the patient’s effort to void, and high postvoid residual volume (incomplete emptying).
FIGURE 4. Cystoscopic view of the urethra showing irregular mucosa overlying a right proximal urethral mass.
FIGURE 5. Histopathology revealing poorly differentiated transitional cell carcinoma of the urethra beneath vaginal epithelium.
nancy. Transurethral biopsies with cold-cup biopsy forceps and transvaginal needle biopsies of the urethral mass should be performed promptly to expedite early treatment of this aggressive malignancy.
The treatment for urethral malignancy is wide local excision for localized disease.6 Distal urethrectomy has been performed for distal disease1; however, in women wide local excision typically requires anterior pelvic exenteration. Adjuvant ra-
436xii
UROLOGY 55 (3), 2000
diation therapy or chemotherapy may be administered for extensive disease or for those patients who are not surgical candidates.5,7 However, despite aggressive therapy for urethral carcinoma, recurrence rates are high and the optimal course of therapy has not been determined. CONCLUSIONS Bladder outlet obstruction in women with no neurologic complaints, no history of pelvic surgery, and no pelvic prolapse should raise the suspicion of urethral malignancy. Urethral malignancy must always be included in the differential diagnosis of any female patient with obstructive symptoms. This is especially true when obstruction exists concomitant with a urethral diverticulum, particularly when accompanied by significant firmness on physical examination, a noncalcified filling defect on voiding cystourethrogram, irregular urethral mucosa or a visible lesion emanating from the communication site on cystoscopy, expression of blood or purulence from the diverticulum, or any degree of hematuria. High clinical suspicion and accurate preoperative diagnosis will
UROLOGY 55 (3), 2000
result in the proper aggressive therapy necessary in treatment of this rare disease. ACKNOWLEDGMENT. To Delver R. Cain, M.D., Pathology, for review of the pathologic studies and other contributions. REFERENCES 1. Gheiler EL, Tefilli MV, Tigert R, et al: Management of primary urethral cancer. Urology 52: 487– 493, 1998. 2. Leach GE, and Trockman BA: Surgery for fistulas and diverticulum, in Walsh PC, Retik AB, Vaughn ED, et al (Eds): Campbell’s Urology, 7th ed. Philadelphia, WB Saunders, 1997, pp 1141–1151. 3. Kobashi KC, and Leach GE: Diverticulum and urethral fistula, in Cardozo L, and Staskin D (Eds): Textbook of Female Urology and Urogynecology. Oxford, United Kingdom, Isis Medical Media, 2000. 4. Evans KJ, McCarthy MP, and Sands JP: Adenocarcinoma of a female urethral diverticulum: case report and review of the literature. J Urol 126(1): 124 –126, 1981. 5. Poore RE, and McCullough DL: Urethral carcinoma, in Gillenwater JY, Grayhack JT, Howards SS, et al (Eds): Adult and Pediatric Urology, 3rd ed. Salem, Mosby, pp 1846 –1847. 6. Palagiri A: Urethral diverticulum with endometriosis. Urology 11: 271–272, 1978. 7. Gonzalez MO, Harrison ML, and Boileau MA: Carcinoma in diverticulum of female urethra. Urology 26: 328 – 332, 1985.
436xiii