LEYDIG CELL TUMORS AND TUMORS ASSOCIATED WITH CONGENITAL ADRENAL HYPERPLASIA

doi:10.1016/S0094-0143(05)70099-9

Urologic Clinics of North America

Volume 27, Issue 3, 1 August 2000, Pages 519-528

https://doi.org/10.1016/S0094-0143(05)70099-9 Get rights and content

Testicular cancers occur at a rate of 2 cases per 100,000 males.⁴⁰ Gonadal stromal tumors, including Leydig cell tumors and tumors of the adrenogenital syndrome, account for 1% to 3% of these neoplasms.6, 27, 52, 54 Despite their rarity, these hormone-producing tumors are particularly interesting because of their potential for causing endocrinologic manifestations in prepubertal and adult males.⁶³ They are also clinically significant, and early identification is critical to avoid profound and often irreversible developmental changes in affected children. An accurate diagnosis is important to differentiate tumors that will respond to medical management from tumors that require definitive surgical therapy.⁴²

Section snippets

LEYDIG CELL ONTOGENY

The ontegeny of Leydig cells is intimately related to the fluxes of testosterone that occur throughout the life of males.23, 29, 36, 48 Leydig cells begin to differentiate from the intermediate mesoderm of the embryo by the eighth week of gestation. At the time of peak testosterone activity, around 15 weeks, Leydig cells occupy more than half of the volume of the testicle. Progressive involution subsequently occurs from week 16 to birth that results in a 60% reduction in Leydig cell volume. A

LEYDIG CELL TUMORS

Leydig cell tumors are rare gonadal stromal tumors first identified about 100 years ago.⁵³ They may occur at any age and represent approximately 40% of all non–germ cell testicular tumors.5, 9 Leydig cell tumors are usually unilateral and benign. In children, they typically occur in boys between the ages of 4 and 10 years and account for about 10% of cases of precocious puberty.¹³

Pathophysiology

The development of testicular masses in patients with the adrenogenital syndrome represents an interesting manifestation of isosexual precocious puberty. A basic understanding of the pathways of steroid hormone synthesis is necessary to appreciate the clinical manifestations of the syndrome (Fig. 2).³⁸ Diminished production of cortisol results from a group of autosomal recessive defects in adrenal steroidogenesis. The most common and classic form of adrenogenital syndrome is caused by deficient

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A middle-aged man with hyperandrogenic state
2023, Urology Case Reports
Hyperandrogenism secondary to testicular cancer typically arises in patients in whom Leydig cell hyperplasia or neoplasia can be identified. Additionally, benign and malignant adrenocortical tumors can also present with signs and symptoms of hyperandrogenism. We report a case of a 40-year-old gentleman who experienced several months of weight gain, worsening gynecomastia, and mood changes secondary to high testosterone and estradiol levels. Workup initially was negative for testicular malignancy and positive for a benign-appearing lesion in the adrenal gland. Despite adrenalectomy, symptoms continued to persist and ultimately a testicular cancer without Leydig cell involvement was identified.
Ultrasonography of the scrotum: Revisiting a classic technique
2021, European Journal of Radiology
Conventional US is the primary imaging modality for the evaluation of the scrotum, due to its high resolution, availability, cost-effectiveness and absence of ionizing radiation. Grayscale and color Doppler US provide a comprehensive assessment of scrotal diseases. The technique represents the mainstay for imaging of acute scrotum. US is highly accurate in the detection, localization and characterization of scrotal masses.
Multiparametric US, including conventional US, contrast-enhanced US and tissue elastography has improved the diagnostic performance of the technique in the assessment of testicular diseases. MRI represents a valuable supplemental imaging tool for the investigation of scrotal pathology, mainly recommended in cases of indeterminate US findings.
Recommendations recently issued by the European Society of Urogenital Radiology Scrotal and Penile Imaging Working Group (ESUR-SPIWG) refer to the use of scrotal sonography for the evaluation of pathologic entities, including testicular microlithiasis, small, non-palpable incidentally detected testicular masses, varicocele and scrotal trauma.
In this review, the technical specifications for scrotal US and the normal sonographic findings are presented. Grayscale and color Doppler US findings of common acute scrotal diseases and scrotal masses are discussed. The complimentary role of multiparametric US and scrotal MRI is addressed. ESUR-SPIWG’s guidelines are also reviewed.
Three Cases of Testicular Adrenal Rest Tumors in Congenital Adrenal Hyperplasia—A Diagnostic and Therapeutic Challenge
2019, Urology
Citation Excerpt :
Unlike TARTs histopathology of LCT reveals specific Reinke crystalloids in about 25%-40 %, but their absence does not rule out LCT. When distinguishing between TART and LCT, the following features make diagnosis of TART more likely: young age, bilateral presence of tumors, history of poor therapeutic compliance, and absence of metastasis.6,7 Most importantly, one has to be aware of the fact that the patient has CAH: whereas TARTs occur in a high percentage of CAH patients, LCTs in CAH patients have rarely been described.8,9
Leydig cell testicular tumors: About a case report
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Testicular tumors
2018, Encyclopedia of Endocrine Diseases
This article contains a short overview of biology, genetics and epidemiology of testicular tumors, combined with a list of management options and concerns. Testicular tumors are endocrine-related neoplasms that occur most often in young men and children. Germ cell-derived tumors (TGCT) constitute the vast majority of cases. Somatic tumors are rare, usually caused by gene mutations, and can be a part of syndromes, e.g. Carney complex. Most common TGCT (seminomas and non-seminomas) are derived from a precursor lesion, germ cell neoplasia in situ (GCNIS), which allows early diagnosis. These tumors have been rising in incidence, but the etiology has not been elucidated. Epidemiologic features suggest a combined influence of genetic and environmental factors, the latter acting primarily during early development. Recent genome-wide association studies revealed a number of susceptibility genes, helping in understanding the pathogenesis. TGCT usually have a good prognosis, because of a high sensitivity to radio- and chemotherapy. However, due to the young age of the survivors, preservation of reproductive function, endocrine balance and preventing late effects must be considered by the management team.
Testicular Adrenal Rest Tumor (TART) in congenital adrenal hyperplasia
2017, European Journal of Medical Genetics
Congenital adrenal hyperplasia is one of the most common autosomal recessive genetic disorders. Testicular adrenal tumors are significant complications of congenital adrenal hyperplasia. We would like to present two patients of testicular adrenal rest tumors. Patient 1 24 year-old male, he was diagnosed with congenital adrenal hyperplasia at the age of 8 due to precocious puberty. He received hydro-cortisone treatment until the age of 18. Testicular mass had been detected and right radical orchiectomy had been applied 6 months ago and reported as testicular adrenal rest tumor. In scrotal ultrasound, a mixed type mass lesion (6 × 4x3 cm) covering a large part of left testis was observed. The imaging findings were consistent with adrenal rest tumor. The patient took adrenocorticotropic hormone supressive therapy with dexamethasone 0.75 mg once a day. Patient 2, 38 year-old male, he had been followed-up as adrenal insufficiency for 35 years. He underwent right orchiectomy operation due to the testicular mass in 2010 and the pathological examination revealed Leydig cell tumor. In scrotal ultrasound, small multifocal lesions were detected on the left testis and resection was done. It was reported as testicular adrenal rest tumor. He is being followed-up with glucocorticoid treatment according to androgen and adrenocorticotropic hormone levels. Early diagnosis of testicular adrenal rest tumor is significant in preventing irreversible testicular damage and infertility. In the differential diagnosis, we should keep in mind that testicular adrenal rest tumor can mimic other testicular tumors such as primary germ cell tumors.

View all citing articles on Scopus

: Address reprint requests to: Mark A. Rich, MD, Division Pediatric Urology, Nemours Children Clinic, 83 West Columbia, Orlando, Florida 32806

^*: Department of Pediatric Urology, Nemours Children's Clinic, Orlando Florida

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LEYDIG CELL TUMORS AND TUMORS ASSOCIATED WITH CONGENITAL ADRENAL HYPERPLASIA

Section snippets

LEYDIG CELL ONTOGENY

LEYDIG CELL TUMORS

Pathophysiology

Fertil Steril

J Pediatr

Urology

J Urol

Urol Clin North Am

Clin Endocrinol Metab

J Urol

J Urol

J Pediatr Surg

Biochem Biophys Res Commun

J Pediatr

J Pediatr

J Urol

J Urol

J Urol

Urol Clin North Am

J Pediatr

The effects of prolonged oestrogen administration upon male mice of various strains: Development of testicular tumors in the strong A strain

J Pathol

Les inclusions de tissu surrenalien functionnel dans les gonades

Acta Endocr J Endocr Pitie

Leydig cell tumor of the testis: A clinopathologic, DNA content, and MIB-1 comparison of nonmetastasizing and metastasizing tumors

Am J Surg Pathol

Rodent Leydig cell tumorigenesis: A review of the physiology, pathology, and relevance to humans

Clinical Reviews in Toxicology

Bilateral testicular tumors in congenital adrenal hyperplasia: A continuing diagnostic and therapeutic dilemma

Clin Endocrinol

Transiently elevated levels of c-fos and c-myc oncogene messenger ribonucleic acids in cultured murine Leydig tumor cells after addition of human choronic gonadotropin

Mol Endocrinol

Aberrant adrenal cortical tissue near the testis in human infants

Am J Pathol

Occult testicular interstitial cell tumor in a patient with gynaecomastia: Ultrasound detection

Radiology

Pituitary and hypothalamus: Perspectives and overviews

Current management of congenital adrenal hyperplasia (CAH): A survey of the Lawson Wilkins Pediatric Endocrine Society (LWPES)

Pediatr Res

Evidence of testicular activity in early infancy

J Clin Endocrinol Metab

Feminizing interstitial cell tumor of the testis: Personal observations and review of the literature

Cancer

Metastatic Leydig cell tumor of the testis: Report of three cases and review of the literature

Cancer Res

Activation of testicular adrenal rest tissue by prolonged excessive ACTH production

J Clin Endocrinol Metab

Hormonal control of Leydig cell differentiation

Ann NY Acad Sci