Prostate gland development and adrenal tumor in a female with congenital adrenal hyperplasia: A case report and review from radiology perspective

Abstract

We describe a case of a female with simple virilizing congenital adrenal hyperplasia (CAH) reared as a male diagnosed at the late age of 64. Computed Tomography (CT) demonstrated a large adrenal mass, bilateral diffuse adrenal enlargement, female pelvic organs as well as a clearly visualized prostate gland. This is to the best of our knowledge the first case of such a sizable prostate gland in a female CAH patient documented on CT. We review the literature regarding aspects where radiologists may encounter CAH and the finding of presence of a prostate gland in female CAH patients.

Prostatic tissue in 46XX congenital adrenal hyperplasia: Case report and literature review

“…Calcifications found inside the prostatic tissue in our case were reported only once in a 64-year-old phenotypic male with 46XX karyotype. 16 PSA dosage and transrectal biopsy of the prostate are useful to detect adenocarcinoma. This malignant prostate tumor was described twice in 46XX patients with CAH due to 21-OHD.…”

“…14 Adrenal masses are common in CAH, up to 82% in homozygous and 45% in heterozygous patients. 16 The size of both adrenal glands and tumors has been described to be significantly correlated with a poor hormonal control status, and it can regress with adequate treatment. 17 In our case, the long evolution of the disease without medications controlling hormonal parameters could explain the large size of the adrenal mass.…”

“…4,5 Female patients with CAH having prostatic tissue found in the literature are described in Table 2. [6][7][8][9][10][11][12][13][14][15][16][17] To the best of our knowledge, this is the 20th reported case.…”

A comparison of prostatic development in xenografts of human fetal prostate and human female fetal proximal urethra grown in dihydrotestosterone-treated hosts

“…The idea of prostatic development in xenografts of human fetal female proximal urethra can be inferred from studies in mice in which prostate can be induced when female urogenital sinuses are grafted into male hosts (Cunha, 1975) and in earlier animal studies in which female urogenital sinus formed prostatic buds when cultured in the presence of testosterone (Price and Ortiz, 1965;Takeda et al, 1986). Further support for prostatic development from human fetal urogenital sinus comes from radiographic evidence of prostate in a genetic XX female patient secondary to physiologic elevation in androgens due to congenital adrenal hyperplasia (Fang et al, 2013). Prostatic carcinogenesis has also been reported in genetic XX female patients also with congenital adrenal hyperplasia (Wesselius et al, 2017;Winters et al, 1996).…”

A Patient (46XX) With Congenital Adrenal Hyperplasia and Prostate Cancer: A Case Report

“…The presence of prostate tissue in female patients with CAH has been described in a limited number of cases [ 2 ]. The Skene gland, a para -urethral gland in females homologous to the prostate gland [ 3 ], can develop into tissue that is histologically identical to the male prostate in the presence of androgen excess [ 1 ].…”