869
Aur. N.Z.J . Surg. 1991,61,869-871
ADENOCARCINOMA IN A FEMALE URETHRAL DIVERTICULUM R. B. THOMAS'*AND B. MAGUIRE~~ Departments of *Urology and 'Histopathology , Fremantle Hospital, Fremantle, WesternAustralia Carcinoma occumng within a diverticulum of the female urethra is an exceedingly rare disease for which the treatment is ill-defined. We present here the fmt reported case of this condition from Australasia and review some of the recommendations for treatment.
Key words. carcinoma,cysto-urethredomy,diverticulum, radiotherapy,uretbra.
Introduction Adenocarcinoma of the female urethra is a rare disease. The occurrence of adenocarcinoma within a urethral diverticulum is rarer still. The first reported case of this condition was in 1951' and to date only 21 other cases have been r e p ~ r t e d . ~ A small number of review articles have appeared in the past 20 years, providing varying recommendations for treatment. Because of the rarity of these lesions, a low index of suspicion for the disease exists and they may be allowed to progress before investigation and treatment are considered. We present here a single case of this rare tumour and review the recommendations for therapy. Case report
A 58 year old woman was referred to our outpatient department because of her sensation of incomplete voiding. Physical examination showed a cystic swelling immediately adjacent to the anterior vaginal wall. Ultrasound of the pelvis revealed a 4cm spherical mass immediately posterior to the urethra (Fig. 1). The mass showed a homogeneous echo pattern. A post-voiding ultrasound revealed incomplete bladder emptying. Digital compression of the mass produced extrusion of copious quantities of a mucus-like material from the urethral meatus. No other signs or symptoms could be elicited. A presumptive diagnosis of a simple urethral diverticulum was made and trans-vaginal urethral diverticulectomy was undertaken. Histopathology of the diverticulum showed a moderately differentiated adenocarcinoma with malignancy extending to the mouth of the diverticulum. I
MB. BS; Urology Registrar. 'MB. BS; Pathology Registrar.
Correspondence:R. B. Thomas, Gordon Craig Fellow, Dewment of Surgery,Sydney University, Sydney, NSW 2006. Australia. Accepted for publication 21 November 1990.
In view of the probability of residual tumour, the patient's otherwise good health and the absence of detectable spread on abdominal and pelvic computerized tomography (CT)and plain chest X-ray, a radical cysto-urethrectomy was undertaken. -0gY
The resected specimens consisted of urinary bladder, urethra, uterus and diverticulum. The diverticulum was filled with much and its thickness varied up to a maximum of 3 mm.
Macroscopic appearance The urethra was f m and appeared swollen. On sectioning, its entire length was involved by a 5 cm long and 2.5 cm diameter tumour. Tumour extended through the wall of the urethra and up to the bladder neck in the submucosal plane. Posteriorly, it extended close to the anterior vaginal wall.
Microscopy Samples from the urethral tumour showed numerous collections of malignant epithelial cells infiltrating the whole thickness of the wall, including the muscularis propria. The tumour cells were tall columnar or signet ring in morphology, and actively mucin-secreting; in most of the tumour they were seen in small groups within lakes of mucin (Fig. 2). The cell nuclei showed a moderate degree of pleomorphism and hyperchromasia. The tumour cells showed positive staining for carcino-embryonic antigen. Sections including the wall of the diverticulum showed it to be lined by mucus-secreting columnar epithelium (Fig. 3). The urethral mucosa consisted mainly of transitional epithelium with focal areas of cuboidal epithelium. In some places the mucosal connective tissue showed infiltration by tumour. Samples from the vaginal wall showed infiltrating &nocarcinoma.
THOMAS AND MAGUIRE
870
Fig. 1. (a) Sagittal and (b)coronal ultrasound scans showing a 4cm homogeneous mass anterior to the bladder consistent with a urethral diverticulum.
Fig. 3. Part of urethral diverticulum showing a section of
mucin-secreting columnar epithelium, resembling colonic epithelium. In one area to the left, malignant cells can be seen within mucin lakes in the lamina propria ( X 2 5 ) . Fig. 2. Mucin-secretingcells lying within lakes of mucus with the muscularis propria of the urethral wall ( X 33).
Discussion Only 40 cases of urethral diverticular carcinoma have been reported to date. The literature u until 1985 has been reviewed by Gonzales et (11. They noted that, while carcinomas of the urethra proper were usually squamous, 40-50% of those arising within a diverticulum were adenocarcinomas. The age at diagnosis ranged from 42 to 76 years, with a mean of 5 I years. Of the 40 reported cases, twentyone were adenocarcinomas and the remainder were either transitional cell, squamous cell or carcinoma
P
in siru.' The pathology of at least seven cases is
unknown, as were only been mentioned as a personal communication in a review by Wishard et The origin of these adenocarcinomas is unclear. Their possible origin from Skenes glands, urethritis glandularis or cloacogenic rests has been postulated by a number of authors.s.6 The investigation of these lesions has been made in a number of ways, including CT, ultrasound, urethrography and cystoscopy, emphasizing the heterogeneity of symptoms with which these patients present.' The most common symptoms that have been noted include dysuria and frequency,' urethral bleeding and outflow obstruction.
87 I
ADENOCARCINOMA OF THE FEMALE URETHRA
Because of the small number of reported cases and a lack of adequate staging criteria there has been significant non-uniformity in the management of these cases. Local excision alone (diverticulectomy) has been advocated by Evans et a / ., based on a review of 12 cases where the tumour appeared confined to the diverticul~m.~ The average followup in this series, however, was less than 18 months and four of their patients suffered a local recurrence within this time. Similarly, reports of high local recurrence rates of 55% by Patanaphan et al. and 66% by Tesluk et a / . emphasize that diverticulectomy alone cannot be recommended as definitive Gonzalez et a / . reported that diverticulectomy combined with external beam radiotherapy (2000-5000 rad) and interstitial radiotherapy (3000-4000 rad) produced a 10-year disease-free survival in two patients who both had a low grade cytological appearance to their tumours. The same treatment in four patients with a more. unfavourable cytological appearance resulted in three patients succumbing within 3 years. Radical cysto-urethrectomy has been reported in three patients by Patanaphan et a / . to give a similiar survival rate to that of local excision and radiotherapy (6O0/o) although, unfortunately, their period of follow-up was not specified.' Other reports of cysto-urethrectomy have in general not included adequate follow-up data to allow comparison of survival rates obtained with radiotherapy. Any recommendations for treatment of this disease must be regarded with caution in view of the small patient numbers that have been reported and the lack of staging criteria for these lesions. Our recommendation is for either cysto-urethrectomy or local excision combined with both external beam and interstitial radiotherapy, as it is obvious that these tumours may be aggressive and that, apart from the possible association of low cytological grade with a favourable outcome, we have no reli-
'
able criteria on which to base a prognosis. The rarity of these lesions highlights a need for an international registry for such tumours to facilitate more accurate comparison between grading, stage and treatment. Finally, an index of suspicion for tumour should be maintained prior to considering simple diverticulectomy for any urethral diverticulum in a female. Our patient is now 20 months postoperative and, at this early stage, is seemingly free of disease.
Acknowledgement The authors thank Mr A. G. S. Tulloch for his encouragement and details of the case.
References I . GONZALEZ M., HARRISON M. & BOILEAU M. (1985) Carcinoma in diverticulum of female urethra. Urology 24. 2. HAMILTON J. D. & LEACH W. B. (1951) Adenocarcinoma arising in a diverticulum of the female urethra. Arch. Parhol. 51, 90. 3. TESLUK H. (1981) Primary adenocarcinoma of female urethra associated with diverticula. Urology 17, 197-9. 4. MARHSALL S. & HIRSCH K. (1977) Carcinoma within urethral diverticula. Urolugy 2, 161-3. 5. TINESS. C . , BICONGIARI L. & WEIGEL J. (1982) Carcinoma in diverticulum of female urethra. Amer. J. Rad. 138. 582-5. 6. EVANS K. I., MCCARTHY M. P. & SANDSJ . P. (1981) Adenocarcinoma of a female urethral diverticulum. A case report and review of the literature. J. Urol. 126, 124-6. 7. PATANAPHAN V., PREMPREE T. er a/. (1983) Adenocarcinoma arising in female urethral diverticulum. Urology 22,259-63. 8 . WISHARD W.N. JR, NOURSE M. H. & MERTZ J. H. 0. (1963) Carcinoma in a diverticulum of the female urethra. J. Urol. 8 9 . 4 3 1.
Aur. N.Z.J . Surg. 1991,61,869-871
ADENOCARCINOMA IN A FEMALE URETHRAL DIVERTICULUM R. B. THOMAS'*AND B. MAGUIRE~~ Departments of *Urology and 'Histopathology , Fremantle Hospital, Fremantle, WesternAustralia Carcinoma occumng within a diverticulum of the female urethra is an exceedingly rare disease for which the treatment is ill-defined. We present here the fmt reported case of this condition from Australasia and review some of the recommendations for treatment.
Key words. carcinoma,cysto-urethredomy,diverticulum, radiotherapy,uretbra.
Introduction Adenocarcinoma of the female urethra is a rare disease. The occurrence of adenocarcinoma within a urethral diverticulum is rarer still. The first reported case of this condition was in 1951' and to date only 21 other cases have been r e p ~ r t e d . ~ A small number of review articles have appeared in the past 20 years, providing varying recommendations for treatment. Because of the rarity of these lesions, a low index of suspicion for the disease exists and they may be allowed to progress before investigation and treatment are considered. We present here a single case of this rare tumour and review the recommendations for therapy. Case report
A 58 year old woman was referred to our outpatient department because of her sensation of incomplete voiding. Physical examination showed a cystic swelling immediately adjacent to the anterior vaginal wall. Ultrasound of the pelvis revealed a 4cm spherical mass immediately posterior to the urethra (Fig. 1). The mass showed a homogeneous echo pattern. A post-voiding ultrasound revealed incomplete bladder emptying. Digital compression of the mass produced extrusion of copious quantities of a mucus-like material from the urethral meatus. No other signs or symptoms could be elicited. A presumptive diagnosis of a simple urethral diverticulum was made and trans-vaginal urethral diverticulectomy was undertaken. Histopathology of the diverticulum showed a moderately differentiated adenocarcinoma with malignancy extending to the mouth of the diverticulum. I
MB. BS; Urology Registrar. 'MB. BS; Pathology Registrar.
Correspondence:R. B. Thomas, Gordon Craig Fellow, Dewment of Surgery,Sydney University, Sydney, NSW 2006. Australia. Accepted for publication 21 November 1990.
In view of the probability of residual tumour, the patient's otherwise good health and the absence of detectable spread on abdominal and pelvic computerized tomography (CT)and plain chest X-ray, a radical cysto-urethrectomy was undertaken. -0gY
The resected specimens consisted of urinary bladder, urethra, uterus and diverticulum. The diverticulum was filled with much and its thickness varied up to a maximum of 3 mm.
Macroscopic appearance The urethra was f m and appeared swollen. On sectioning, its entire length was involved by a 5 cm long and 2.5 cm diameter tumour. Tumour extended through the wall of the urethra and up to the bladder neck in the submucosal plane. Posteriorly, it extended close to the anterior vaginal wall.
Microscopy Samples from the urethral tumour showed numerous collections of malignant epithelial cells infiltrating the whole thickness of the wall, including the muscularis propria. The tumour cells were tall columnar or signet ring in morphology, and actively mucin-secreting; in most of the tumour they were seen in small groups within lakes of mucin (Fig. 2). The cell nuclei showed a moderate degree of pleomorphism and hyperchromasia. The tumour cells showed positive staining for carcino-embryonic antigen. Sections including the wall of the diverticulum showed it to be lined by mucus-secreting columnar epithelium (Fig. 3). The urethral mucosa consisted mainly of transitional epithelium with focal areas of cuboidal epithelium. In some places the mucosal connective tissue showed infiltration by tumour. Samples from the vaginal wall showed infiltrating &nocarcinoma.
THOMAS AND MAGUIRE
870
Fig. 1. (a) Sagittal and (b)coronal ultrasound scans showing a 4cm homogeneous mass anterior to the bladder consistent with a urethral diverticulum.
Fig. 3. Part of urethral diverticulum showing a section of
mucin-secreting columnar epithelium, resembling colonic epithelium. In one area to the left, malignant cells can be seen within mucin lakes in the lamina propria ( X 2 5 ) . Fig. 2. Mucin-secretingcells lying within lakes of mucus with the muscularis propria of the urethral wall ( X 33).
Discussion Only 40 cases of urethral diverticular carcinoma have been reported to date. The literature u until 1985 has been reviewed by Gonzales et (11. They noted that, while carcinomas of the urethra proper were usually squamous, 40-50% of those arising within a diverticulum were adenocarcinomas. The age at diagnosis ranged from 42 to 76 years, with a mean of 5 I years. Of the 40 reported cases, twentyone were adenocarcinomas and the remainder were either transitional cell, squamous cell or carcinoma
P
in siru.' The pathology of at least seven cases is
unknown, as were only been mentioned as a personal communication in a review by Wishard et The origin of these adenocarcinomas is unclear. Their possible origin from Skenes glands, urethritis glandularis or cloacogenic rests has been postulated by a number of authors.s.6 The investigation of these lesions has been made in a number of ways, including CT, ultrasound, urethrography and cystoscopy, emphasizing the heterogeneity of symptoms with which these patients present.' The most common symptoms that have been noted include dysuria and frequency,' urethral bleeding and outflow obstruction.
87 I
ADENOCARCINOMA OF THE FEMALE URETHRA
Because of the small number of reported cases and a lack of adequate staging criteria there has been significant non-uniformity in the management of these cases. Local excision alone (diverticulectomy) has been advocated by Evans et a / ., based on a review of 12 cases where the tumour appeared confined to the diverticul~m.~ The average followup in this series, however, was less than 18 months and four of their patients suffered a local recurrence within this time. Similarly, reports of high local recurrence rates of 55% by Patanaphan et al. and 66% by Tesluk et a / . emphasize that diverticulectomy alone cannot be recommended as definitive Gonzalez et a / . reported that diverticulectomy combined with external beam radiotherapy (2000-5000 rad) and interstitial radiotherapy (3000-4000 rad) produced a 10-year disease-free survival in two patients who both had a low grade cytological appearance to their tumours. The same treatment in four patients with a more. unfavourable cytological appearance resulted in three patients succumbing within 3 years. Radical cysto-urethrectomy has been reported in three patients by Patanaphan et a / . to give a similiar survival rate to that of local excision and radiotherapy (6O0/o) although, unfortunately, their period of follow-up was not specified.' Other reports of cysto-urethrectomy have in general not included adequate follow-up data to allow comparison of survival rates obtained with radiotherapy. Any recommendations for treatment of this disease must be regarded with caution in view of the small patient numbers that have been reported and the lack of staging criteria for these lesions. Our recommendation is for either cysto-urethrectomy or local excision combined with both external beam and interstitial radiotherapy, as it is obvious that these tumours may be aggressive and that, apart from the possible association of low cytological grade with a favourable outcome, we have no reli-
'
able criteria on which to base a prognosis. The rarity of these lesions highlights a need for an international registry for such tumours to facilitate more accurate comparison between grading, stage and treatment. Finally, an index of suspicion for tumour should be maintained prior to considering simple diverticulectomy for any urethral diverticulum in a female. Our patient is now 20 months postoperative and, at this early stage, is seemingly free of disease.
Acknowledgement The authors thank Mr A. G. S. Tulloch for his encouragement and details of the case.
References I . GONZALEZ M., HARRISON M. & BOILEAU M. (1985) Carcinoma in diverticulum of female urethra. Urology 24. 2. HAMILTON J. D. & LEACH W. B. (1951) Adenocarcinoma arising in a diverticulum of the female urethra. Arch. Parhol. 51, 90. 3. TESLUK H. (1981) Primary adenocarcinoma of female urethra associated with diverticula. Urology 17, 197-9. 4. MARHSALL S. & HIRSCH K. (1977) Carcinoma within urethral diverticula. Urolugy 2, 161-3. 5. TINESS. C . , BICONGIARI L. & WEIGEL J. (1982) Carcinoma in diverticulum of female urethra. Amer. J. Rad. 138. 582-5. 6. EVANS K. I., MCCARTHY M. P. & SANDSJ . P. (1981) Adenocarcinoma of a female urethral diverticulum. A case report and review of the literature. J. Urol. 126, 124-6. 7. PATANAPHAN V., PREMPREE T. er a/. (1983) Adenocarcinoma arising in female urethral diverticulum. Urology 22,259-63. 8 . WISHARD W.N. JR, NOURSE M. H. & MERTZ J. H. 0. (1963) Carcinoma in a diverticulum of the female urethra. J. Urol. 8 9 . 4 3 1.
