Biomaterial Database

Congenital adrenal hyperplasia with testicular tumors, aggression, and gonadal failure. 1993

E J Keely, and I Matwijiw, and J A Thliveris, and C Faiman

Department of Medicine, University of Manitoba, Winnipeg, Canada.

Bilateral testicular tumors (adrenal rests) may occur in untreated or poorly controlled congenital adrenal hyperplasia. This case report describes two unique associated phenomena: (1) psychologic disturbances similar to those seen with exogenous androgen abuse, which resolved with appropriate glucocorticoid suppression of androgen over-production by this abnormal adrenal/adrenal rest tissue; and (2) testicular failure which showed a partial, delayed recovery with corticosteroid therapy. The need for a careful history and biochemical screening for all patients with bilateral testicular tumors is reinforced.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000312	Adrenal Hyperplasia, Congenital	A group of inherited disorders of the ADRENAL GLANDS, caused by enzyme defects in the synthesis of cortisol (HYDROCORTISONE) and/or ALDOSTERONE leading to accumulation of precursors for ANDROGENS. Depending on the hormone imbalance, congenital adrenal hyperplasia can be classified as salt-wasting, hypertensive, virilizing, or feminizing. Defects in STEROID 21-HYDROXYLASE; STEROID 11-BETA-HYDROXYLASE; STEROID 17-ALPHA-HYDROXYLASE; 3-beta-hydroxysteroid dehydrogenase (3-HYDROXYSTEROID DEHYDROGENASES); TESTOSTERONE 5-ALPHA-REDUCTASE; or steroidogenic acute regulatory protein; among others, underlie these disorders.	Congenital Adrenal Hyperplasia,Hyperplasia, Congenital Adrenal,Adrenal Hyperplasias, Congenital,Congenital Adrenal Hyperplasias,Hyperplasias, Congenital Adrenal
D000314	Adrenal Rest Tumor	Neoplasm derived from displaced cells (rest cells) of the primordial ADRENAL GLANDS, generally in patients with CONGENITAL ADRENAL HYPERPLASIA. Adrenal rest tumors have been identified in TESTES; LIVER; and other tissues. They are dependent on ADRENOCORTICOTROPIN for growth and adrenal steroid secretion.	Adrenal Cortical Rest Tumor,Adrenal Rest Tumors,Rest Tumor, Adrenal,Rest Tumors, Adrenal,Tumor, Adrenal Rest,Tumors, Adrenal Rest
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults
D000374	Aggression	Behavior which may be manifested by destructive and attacking action which is verbal or physical, by covert attitudes of hostility or by obstructionism.	Aggressions
D013736	Testicular Neoplasms	Tumors or cancer of the TESTIS. Germ cell tumors (GERMINOMA) of the testis constitute 95% of all testicular neoplasms.	Cancer of Testis,Cancer of the Testes,Testicular Cancer,Testicular Neoplasm,Testicular Tumor,Testis Cancer,Cancer of the Testis,Neoplasms, Testicular,Neoplasms, Testis,Testicular Tumors,Testis Neoplasms,Tumor of Rete Testis,Cancer, Testicular,Cancer, Testis,Cancers, Testicular,Cancers, Testis,Neoplasm, Testicular,Neoplasm, Testis,Rete Testis Tumor,Rete Testis Tumors,Testicular Cancers,Testis Cancers,Testis Neoplasm,Testis Tumor, Rete,Testis Tumors, Rete,Tumor, Testicular,Tumors, Testicular
D013737	Testis	The male gonad containing two functional parts: the SEMINIFEROUS TUBULES for the production and transport of male germ cells (SPERMATOGENESIS) and the interstitial compartment containing LEYDIG CELLS that produce ANDROGENS.	Testicles,Testes,Testicle