INTRODUCTION
Primary urethral cancer in females is rare, with annual overall incidence rate of 1.5 million. We report on a case of advanced proximal urethral carcinoma treated with surgical resection.
CASE
A 61 year old female, presents with one month history of gross hematuria. Physical examination showed a submucosal anterior vaginal wall mass. Cystoscopy with biopsy showed a proximal urethral mass that was consistent with clear cell adenocarcinoma. PET/CT scan showed a hypermetabolic mass within the vaginal vault. The patient was admitted and underwent anterior pelvic extenteration, bilateral pelvic lymph node dissection. The patient was seen 6 months post-op, has no complaints and is doing well.
PET/CT scan
A focus of increased FDG uptake (SUV up to 17.7) is noted within the vaginal vault. Involvement of the urinary bladder wall is difficult to ascertain due to the presence of physiologic FDG in the urine in the bladder.
DISCUSSION
The differentials for the urethral mass include clear cell carcinoma of the urethra, clear cell carcinoma of the female genital tract, and urothelial carcinoma. Morphologically, the three differentials look similar. To distinguish the three, PAX8 and p63 immunostains were done. The mass was PAX8 positive and p63 negative. With these findings, urothelial carcinoma is ruled out.
In this section of the anterior vaginal wall, the tumor is noted from the serosal surface extending into the vaginal wall without invading up to the submucosa and mucosa. In another section, the tumor arises from the urethra which shows the transition from transitional urothelium to metaplastic tall columnar epithelium. With this, we are able to rule out clear cell carcinoma of the female genitourinary tract.
Anterior extenteration with pelvic lymph node dissection showed urethral carcinoma, clear cell adenocarcinoma variant, 5.5 cm in greatest dimension, invades up to the urinary bladder neck and anterior vaginal wall but not into the vaginal wall submucosa, positive for lymphovascular space invasion. The urethral margin, vaginal cuff, bilateral ureteral margins, and all surgical lines of resection were negative for tumor. Pathologic staging of the disease is pT3N0M0.
Data on treatment has been few and has been based on single center retrospective case series and extrapolation from treatments and from carcinoma in the urinary bladder.
The patient underwent anterior pelvic exenteration without chemo-or-radiotherapy because of the relatively young age of the patient who presented with hematuria with a biopsy showing an aggressive tumor. Immediate symptom relief and curative intent for an aggressive tumor was the primary goal of the surgeon.
CONCLUSION
Urethral clear cell adenocarcinoma is a rare malignancy. Treatment has been based on small case series in single institutions. Surgery was shown to have good outcome for advanced disease.
