title: Clear cell adenocarcinoma of the urethra: review of the literature.
abstract: 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.
  
  
  OBJECTIVE
  To review the literature on CCAU.
  
  
  METHODS
  Various internet databases were used.
  (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, gynaecologists, 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.
authors: North Manchester General Hospital, Department of Urology, Manchester, UK.
authors: Anthony Kodzo-Grey
authors: AK
authors: Venyo
mesh_terms: D018262:Adenocarcinoma, Clear Cell / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D003131:Combined Modality Therapy; D003937:Diagnosis, Differential; D006801:Humans; D011379:Prognosis; D016896:Treatment Outcome; D014523:Urethral Neoplasms / Q000175:diagnosis / Q000401:mortality / Q000628:therapy