open access: CA125-producing Clear Cell Adenocarcinoma Arising From the Upper Ureter and Renal Pelvis

CA125-producing Clear Cell AdenocarcinomaArising From the Upper Ureter and Renal Pelvis

Clear cell adenocarcinomas similar to those found in the female genital organs can arise in the lower urinary tract of
both women and men. Clear cell adenocarcinomas occurring in the upper urinary system are exceedingly rare. Here, we
present a case of clear cell adenocarcinoma arising from the upper ureter and renal pelvis of a postmenopausal woman
with a ureteral stone. The patient had elevated serum levels of cancer antigen (CA) 125 (103.80 U/mL) and CA19-9
(151.96 U/mL). The tumor showed typical features of tubulopapillary structures lined with clear-to-eosinophilic cytoplasm
and frequent hobnail configuration. The tumor cells were immunoreactive for cytokeratin 7, cytokeratin 20, carcinoembryonic
antigen and CA125, but negative for PAX-2 and α-methylacyl coenzyme A racemase. Given the presence of intestinal
and squamous metaplasia of the adjacent urothelium, we propose that this clear cell adenocarcinoma developed
through a metaplastic process. The tumor behaved so aggressively that the patient developed multiple metastases and
died of the disease 5 months after radical nephroureterectomy. [J Chin Med Assoc 2010;73(1):40–43]