Brief Overview of Clear Cell Adenocarcinoma of the Urethra: Review of the Literature

Background: Clear cell adenocarcinoma of the urethra (CCAU) is extremely rare and a number of
clinicians may be unfamiliar with its diagnosis and biological behaviour.
Aims: To review the literature on CCAU.
Methods: Various internet databases were used.
Results/Literature Review: (i) CCAU occurs in adults and in women in the great majority of cases.
(ii) It has a particular association with urethral diverticulum, which has been present in 56% of the
patients; is indistinguishable from clear cell adenocarcinoma of the female genital tract but is not
associated with endometriosis; and probably does not arise by malignant transformation of
nephrogenic adenoma. (iii) It is usually, readily distinguished from nephrogenic adenoma because of
greater cytological a-typicality and mitotic activity and does not stain for prostate-specific antigen or
prostatic acid phosphatase. (iv) It has been treated by anterior exenteration in women and cystoprostatectomy
in men and at times by radiotherapy; chemotherapy has rarely been given. (v) CCAU is
aggressive with low 5-year survival rates. (vi) There is no consensus opinion of treatment options that
would improve the prognosis.
Conclusions: Few cases of CCAU have been reported. Urologists, gynecologists, pathologists, and
oncologists should report cases of CCAU they encounter and enter them into a multicentric trial to
determine the best treatment options that would improve the prognosis.