Adrenal Rests in the Uro-genital Tract of an Adult Population

Abstract

Ectopic adrenal rests are a rare condition which can be found in various sites, generally in the retroperitoneum or pelvis along the path of gonadal descent. Their real prevalence is unknown. Males are more commonly affected, at least in the pediatric age. Adrenal rests are usually clinically silent and incidentally found in surgical samples, mostly in the pediatric population, and rarely in adults. With the aim of increasing knowledge and estimating the prevalence of ectopic adrenocortical tissue in the adult population, 44 adrenal rests in the urogenital tract of 40 adults are described. These represent approximately 0.07% of the total number of urogenital and gynecological surgeries performed in the 22 considered years. Adrenal rests were identified in the spermatic cord (10 males) and in paraovarian, parasalpingeal, or infundibulopelvic ligament locations (30 females). All but one was incidental findings. One case regarded an adrenocortical carcinoma arisen in adrenal rests. A literature review of adrenal ectopia in the urogenital tract of adults identified 57 reported cases from 53 patients, with similar clinicopathological features as those of our series, with the exception of a lower incidence of parasalpingeal locations. Despite their limited clinical implications, awareness of ectopic adrenal rests is essential also in adults for at least two reasons: (a) to correctly identify sources of adrenocortical hormone production in case of adrenal insufficiency or hormonal imbalance and (b) to avoid misinterpretations in the diagnostic workup of renal cell carcinoma, adrenocortical tumors, and rare gonadal neoplasms, including Sertoli/Leydig cell tumors.

Overview of the 2022 WHO Classification of Adrenal Cortical Tumors

“…In surgical pathology, most adrenal rests are discovered as incidental microscopic findings in various surgical specimens including kidneys or gonads (particularly, spermatic cord [22], rete testis [23][24][25], epididymis [26], ovary/paraovarian [22,[27][28][29][30][31][32][33], salpinx and parasalpinx [22,34,35] ). A recent surgical series from adults identified ectopic adrenal rests in both males and females with a more frequent Fig.…”

“…2 Sporadic nodular adrenocortical disease. This photomicrograph illustrates a sporadic nodular adrenocortical disease The green circle outlines the lesion presentation in para-ovarian tissues [22]. Rare locations of adrenal rests include the placenta [36], broad ligament [22,37,38], retroperitoneum [39,40], urinary bladder and prostate [41], groin [42,43], choroid plexus [44], spine [45][46][47], and lung [48,49].…”

“…This photomicrograph illustrates a sporadic nodular adrenocortical disease The green circle outlines the lesion presentation in para-ovarian tissues [22]. Rare locations of adrenal rests include the placenta [36], broad ligament [22,37,38], retroperitoneum [39,40], urinary bladder and prostate [41], groin [42,43], choroid plexus [44], spine [45][46][47], and lung [48,49]. Among various locations, adrenal cortical choristomas have also been described within TPIT-lineage pituitary neuroendocrine tumors (corticotroph PitNETs) [50,51].…”

“…Adrenal cortical rests and choristomas share immunohistochemical features of adrenal cortical cells as they express SF1 and other non-specific biomarkers of adrenal cortical differentiation [22,51,59,60]. The distinction of adrenal cortical rest-related proliferations from other steroidogenic neoplasms poses a diagnostic challenge and may require the use of adrenal cortex-related steroidogenic enzyme immunohistochemistry as discussed in Question 10.…”

Histopathologic Features of Adrenal Cortical Carcinoma

“…37,78 They are helpful also in defining ACC of ectopic adrenal tissue and in metastases. 79 In particular, SF-1 emerged as the most reliable, with a diagnostic sensitivity and specificity of 98% and 100%, respectively. 33,53,[80][81][82][83][84][85][86][87] In addition, ACC can variably express mesothelial (calretinin and D2-40) and neuronal (synaptophysin and neurofilaments) markers and is negative for epithelial membrane antigen, carcinoembryonic antigen, and chromogranin A.…”