Clear Cell Adenocarcinoma in Men

Abstract

Clear cell adenocarcinoma (CCA) is a rare tumor in the genitourinary tract with female predominance and few reports in men. We identified 15 cases of CCA in men evaluated at our institution. Five arose in the bladder, 7 in the prostate or prostatic urethra, 2 in the membranous urethra (1 multifocal in the prostatic and membranous urethra), 1 periprostatic (likely from an embryologic remnant), and 1 between rectum and bladder (likely in a prostatic utricle cyst). No cases showed associated Müllerian structures. One case showed separate foci of nephrogenic adenoma at diagnosis, and 1 case showed urothelial carcinoma in situ on a later follow-up biopsy. Four tumors extended into other organs (prostate to seminal vesicle and periprostatic soft tissue, periprostatic soft tissue to prostate, prostatic urethra to bladder and rectum, and prostate to bladder neck). One tumor showed extraprostatic extension alone. Four tumors metastasized to lymph nodes, with 3 also metastasizing to other sites (bladder, lung and adrenal, and right flank). Eleven patients underwent resection, including 3 transurethral resections. Seven underwent other treatments, including radiation (5 [1 for recurrence]), chemotherapy (3), hormonal therapy (3), immunotherapy with nivolumab (1), and targeted therapy with gefitinib (1). The mean follow-up was 35 months (range: 1 to 138 mo). At the last follow-up, 7 patients showed no evident disease and 3 were alive with disease. Four died with the cause of death unknown, with 2 cases having confirmed disease at the time of death and the remaining 2 dying less than a year after diagnosis. The mean time to death was 16 months (range: 6 to 39 mo). No follow-up was available on 1 patient. All patients who died in this series had CCA of the prostate or prostatic urethra. Pathologists need to be attuned to CCA occurring in males, given that the literature emphasizes its occurrence in females. In addition to established sites such as bladder and urethra, our series demonstrates that tumor may present in unusual adjacent sites, such as in periprostatic embryologic remnants or prostatic utricle.

Molecular Characterization of Muellerian Tumors of the Urinary Tract

“…They are rare malignancies and occur preferably in women (female-to-male ratio 2:1) or in case of endometrioid adenocarcinomas exclusively in women [1]. However, recently, a series of n = 15 CCAs in men was also reported [4]. Most of the CCAs are located in the bladder neck and trigone, less frequently in the lower posterior bladder wall, whereas EAs are located in the trigone and the posterior wall, where endometriosis is most common [1].…”

Clear cell adenocarcinoma of the prostatic urethra with metachronous prostate adenocarcinoma

“…Mean time to death was 16 months, and all 4 deceased patients had CCA of the prostate or prostatic urethra. 3 …”

“… 1 , 2 Hormone, targeted and immunotherapy have been used in isolated cases. 3 The majority (80%) of CCAU cases underwent operative intervention, most commonly anterior exenteration and pelvic lymph node dissection. 1 , 3 Urethrectomy when performed, occurred in the setting of smaller tumours, and was suggested to be effective.…”

“… 1 It is exceedingly rare in the literature, with as few as 9 cases of CCAU reported to arise within the prostate or prostatic urethra. 2 , 3 …”

GATA3 expression in clear cell adenocarcinoma of the lower urinary tract: a potential diagnostic pitfall

“…Clear cell adenocarcinoma (CCAC) is a malignant neoplasm arising predominantly in the female genital tract (FGT), particularly from the ovary [1] as well as a variant of endometrial carcinoma [2]. CCAC can rarely be seen primarily in the lower urinary tract (LUT) [3], and most commonly occurs in the urethra (particularly in diverticula) and trigone or posterior wall of the urinary bladder [4], with significant female predominance (female-tomale ratio = 3:1) [3,4], although occurrence in male LUT is well documented [5]. CCAC may demonstrate tubulocystic (Fig.…”