Steroidogenic enzyme activities, morphology, and receptor studies of a testicular adrenal rest in a patient with congenital adrenal hyperplasia

Richard V. Clark, Barry D. Albertson, Abraham Munabi, Fernando Cassorla, Greti Aguilera, Dwight W. Warren, Richard J. Sherins, Donald (Lynn) Loriaux

Research output: Contribution to journalArticle

73 Citations (Scopus)

Abstract

Steroid-secreting tumors of the testis have generally been considered to be of Leydig cell origin. Testicular tumors in patients with congenital adrenal hyperplasia have been thought to be adrenal rests, but no conclusive evidence supporting the hypothesis has been presented. We report a morphological and biochemical analysis of a patient with 21-hydroxylase deficiency who developed bilateral nodular hyperplasia of steroid-secreting tissue within the testis, despite suppression therapy with both exogenous glucocorticoids and testosterone. The tissue was formed of confluent nodules of homogenous cells. Electron microscopy showed the cells to have abundant smooth endoplasmic reticulum, well developed Golgi apparatus, and mitochondria with predominantly tubular cristae features characteristic of steroid-secreting cells of adreno-cortical origin. Crystals of Reinke were not observed. Functional studies in vivo showed a marked response to ACTH infusion, with 17-hydroxyprogesterone rising from 56 to 13,500 ng/mL, cortisol from less than 2 to 19 μg/dL, and testosterone from 369 to 629 ng/dL, with an attendant increase in testicular size and pain over 48 h. Receptor studies in vitro revealed no gonadotropin receptors, but abundant angiotensin-II receptors. Enzyme activity analysis in vitro showed undetectable 21-hydroxylase activity and an enzyme profile consistent with adrenocortical cells rather than Leydig cells. Based on these morphological and biochemical findings, we conclude that the nodular steroidogenic tissue that replaced this patient's testes was of adrenal origin The study documents for the first time the development of adrenocortical tumors from adrenal rest tissue within the testis.

Original languageEnglish (US)
Pages (from-to)1408-1413
Number of pages6
JournalJournal of Clinical Endocrinology and Metabolism
Volume70
Issue number5
StatePublished - May 1990
Externally publishedYes

Fingerprint

Congenital Adrenal Hyperplasia
Enzyme activity
Testis
Tissue
Steroid 21-Hydroxylase
Tumors
Leydig Cells
Steroids
Enzymes
Adrenal Rest Tumor
Testosterone
Gonadotropin Receptors
17-alpha-Hydroxyprogesterone
Smooth Endoplasmic Reticulum
Angiotensin Receptors
Mitochondria
Testicular Neoplasms
Golgi Apparatus
Adrenocorticotropic Hormone
Electron microscopy

ASJC Scopus subject areas

  • Biochemistry
  • Endocrinology, Diabetes and Metabolism

Cite this

Steroidogenic enzyme activities, morphology, and receptor studies of a testicular adrenal rest in a patient with congenital adrenal hyperplasia. / Clark, Richard V.; Albertson, Barry D.; Munabi, Abraham; Cassorla, Fernando; Aguilera, Greti; Warren, Dwight W.; Sherins, Richard J.; Loriaux, Donald (Lynn).

In: Journal of Clinical Endocrinology and Metabolism, Vol. 70, No. 5, 05.1990, p. 1408-1413.

Research output: Contribution to journalArticle

Clark, Richard V. ; Albertson, Barry D. ; Munabi, Abraham ; Cassorla, Fernando ; Aguilera, Greti ; Warren, Dwight W. ; Sherins, Richard J. ; Loriaux, Donald (Lynn). / Steroidogenic enzyme activities, morphology, and receptor studies of a testicular adrenal rest in a patient with congenital adrenal hyperplasia. In: Journal of Clinical Endocrinology and Metabolism. 1990 ; Vol. 70, No. 5. pp. 1408-1413.
@article{6c13e0280a0c44adaafd5857735c4f9c,
title = "Steroidogenic enzyme activities, morphology, and receptor studies of a testicular adrenal rest in a patient with congenital adrenal hyperplasia",
abstract = "Steroid-secreting tumors of the testis have generally been considered to be of Leydig cell origin. Testicular tumors in patients with congenital adrenal hyperplasia have been thought to be adrenal rests, but no conclusive evidence supporting the hypothesis has been presented. We report a morphological and biochemical analysis of a patient with 21-hydroxylase deficiency who developed bilateral nodular hyperplasia of steroid-secreting tissue within the testis, despite suppression therapy with both exogenous glucocorticoids and testosterone. The tissue was formed of confluent nodules of homogenous cells. Electron microscopy showed the cells to have abundant smooth endoplasmic reticulum, well developed Golgi apparatus, and mitochondria with predominantly tubular cristae features characteristic of steroid-secreting cells of adreno-cortical origin. Crystals of Reinke were not observed. Functional studies in vivo showed a marked response to ACTH infusion, with 17-hydroxyprogesterone rising from 56 to 13,500 ng/mL, cortisol from less than 2 to 19 μg/dL, and testosterone from 369 to 629 ng/dL, with an attendant increase in testicular size and pain over 48 h. Receptor studies in vitro revealed no gonadotropin receptors, but abundant angiotensin-II receptors. Enzyme activity analysis in vitro showed undetectable 21-hydroxylase activity and an enzyme profile consistent with adrenocortical cells rather than Leydig cells. Based on these morphological and biochemical findings, we conclude that the nodular steroidogenic tissue that replaced this patient's testes was of adrenal origin The study documents for the first time the development of adrenocortical tumors from adrenal rest tissue within the testis.",
author = "Clark, {Richard V.} and Albertson, {Barry D.} and Abraham Munabi and Fernando Cassorla and Greti Aguilera and Warren, {Dwight W.} and Sherins, {Richard J.} and Loriaux, {Donald (Lynn)}",
year = "1990",
month = "5",
language = "English (US)",
volume = "70",
pages = "1408--1413",
journal = "Journal of Clinical Endocrinology and Metabolism",
issn = "0021-972X",
publisher = "The Endocrine Society",
number = "5",

}

TY - JOUR

T1 - Steroidogenic enzyme activities, morphology, and receptor studies of a testicular adrenal rest in a patient with congenital adrenal hyperplasia

AU - Clark, Richard V.

AU - Albertson, Barry D.

AU - Munabi, Abraham

AU - Cassorla, Fernando

AU - Aguilera, Greti

AU - Warren, Dwight W.

AU - Sherins, Richard J.

AU - Loriaux, Donald (Lynn)

PY - 1990/5

Y1 - 1990/5

N2 - Steroid-secreting tumors of the testis have generally been considered to be of Leydig cell origin. Testicular tumors in patients with congenital adrenal hyperplasia have been thought to be adrenal rests, but no conclusive evidence supporting the hypothesis has been presented. We report a morphological and biochemical analysis of a patient with 21-hydroxylase deficiency who developed bilateral nodular hyperplasia of steroid-secreting tissue within the testis, despite suppression therapy with both exogenous glucocorticoids and testosterone. The tissue was formed of confluent nodules of homogenous cells. Electron microscopy showed the cells to have abundant smooth endoplasmic reticulum, well developed Golgi apparatus, and mitochondria with predominantly tubular cristae features characteristic of steroid-secreting cells of adreno-cortical origin. Crystals of Reinke were not observed. Functional studies in vivo showed a marked response to ACTH infusion, with 17-hydroxyprogesterone rising from 56 to 13,500 ng/mL, cortisol from less than 2 to 19 μg/dL, and testosterone from 369 to 629 ng/dL, with an attendant increase in testicular size and pain over 48 h. Receptor studies in vitro revealed no gonadotropin receptors, but abundant angiotensin-II receptors. Enzyme activity analysis in vitro showed undetectable 21-hydroxylase activity and an enzyme profile consistent with adrenocortical cells rather than Leydig cells. Based on these morphological and biochemical findings, we conclude that the nodular steroidogenic tissue that replaced this patient's testes was of adrenal origin The study documents for the first time the development of adrenocortical tumors from adrenal rest tissue within the testis.

AB - Steroid-secreting tumors of the testis have generally been considered to be of Leydig cell origin. Testicular tumors in patients with congenital adrenal hyperplasia have been thought to be adrenal rests, but no conclusive evidence supporting the hypothesis has been presented. We report a morphological and biochemical analysis of a patient with 21-hydroxylase deficiency who developed bilateral nodular hyperplasia of steroid-secreting tissue within the testis, despite suppression therapy with both exogenous glucocorticoids and testosterone. The tissue was formed of confluent nodules of homogenous cells. Electron microscopy showed the cells to have abundant smooth endoplasmic reticulum, well developed Golgi apparatus, and mitochondria with predominantly tubular cristae features characteristic of steroid-secreting cells of adreno-cortical origin. Crystals of Reinke were not observed. Functional studies in vivo showed a marked response to ACTH infusion, with 17-hydroxyprogesterone rising from 56 to 13,500 ng/mL, cortisol from less than 2 to 19 μg/dL, and testosterone from 369 to 629 ng/dL, with an attendant increase in testicular size and pain over 48 h. Receptor studies in vitro revealed no gonadotropin receptors, but abundant angiotensin-II receptors. Enzyme activity analysis in vitro showed undetectable 21-hydroxylase activity and an enzyme profile consistent with adrenocortical cells rather than Leydig cells. Based on these morphological and biochemical findings, we conclude that the nodular steroidogenic tissue that replaced this patient's testes was of adrenal origin The study documents for the first time the development of adrenocortical tumors from adrenal rest tissue within the testis.

UR - http://www.scopus.com/inward/record.url?scp=0025268868&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0025268868&partnerID=8YFLogxK

M3 - Article

C2 - 2335578

AN - SCOPUS:0025268868

VL - 70

SP - 1408

EP - 1413

JO - Journal of Clinical Endocrinology and Metabolism

JF - Journal of Clinical Endocrinology and Metabolism

SN - 0021-972X

IS - 5

ER -