Histopathological and immunophenotypic features of testicular tumour of the adrenogenital syndrome

Wang, Zhuo; Yang, Shicong; Shi, Huijuan; Du, Hong; Xue, Ling; Wang, Liantang; Dong, Yu; Han, Anqin

doi:10.1111/j.1365-2559.2011.03861.x

Cited by 44 publications

(33 citation statements)

References 20 publications

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“…Surgery is also suggested if an LC neoplasm is suspected, as the majority of LCT cases in this age group are benign. Few studies investigated immunohistochemical profiles of TART and LCT, and the currently available markers include synaptophysin, CD56 (both stronger in TART than in LCT) and the androgen receptor (absent in TART but positive in most cases of LCT) (24,25). A recent study, published after our study had been submitted for publication, investigated classical markers of steroidogenesis at mRNA level, and found most markers expressed in TART (10).…”

Section: Discussionmentioning

confidence: 99%

Abundance of DLK1, differential expression of CYP11B1, CYP21A2 and MC2R, and lack of INSL3 distinguish testicular adrenal rest tumours from Leydig cell tumours

Lottrup

Nielsen

Skakkebæk

et al. 2015

European Journal of Endocrinology

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Objective: Testicular adrenal rest tumours (TARTs) are a common finding in patients with congenital adrenal hyperplasia (CAH). These tumours constitute a diagnostic and management conundrum and may lead to infertility. TART cells share many functional and morphological similarities with Leydig cells (LCs), and masses consisting of such cells are occasionally misclassified as malignant testicular tumours, which may lead to erroneous orchiectomy in these patients. Design: In this study, we aimed to investigate the potential of LC developmental markers and adrenal steroidogenic markers in the differential diagnosis of TARTs and malignant LC tumours (LCTs). Methods: We investigated mRNA and protein expression of testicular steroidogenic enzymes; CYP11A1 and HSD3B1/2, markers of adrenal steroidogenesis; CYP11B1, CYP21A2 and ACTH receptor/melanocortin 2 receptor (MC2R), and markers of LC maturation; and delta-like 1 homolog (DLK1) and insulin-like 3 (INSL3) in testicular biopsies with TART, orchiectomy specimens with LCTs and samples from human fetal adrenals. Results: Expression of testicular steroidogenic enzymes was observed in all specimens. All investigated adrenal steroidogenic markers were expressed in TART, and weak reactions for CYP11B1 and MC2R were observed at the protein level in LTCs. TART and fetal adrenals had identical expression profiles. DLK1 was highly expressed and INSL3 not detectable in TART, whereas INSL3 was highly expressed in LCTs. Conclusions: The similar expression profiles in TART and fetal adrenals as well as the presence of classical markers of adrenal steroidogenesis lend support to the hypothesis that TART develops from a displaced adrenal cell type. Malignant LCTs seem to have lost DLK1 expression and do not resemble immature LCs. The different expression pattern of DLK1, INSL3 and most adrenocortical markers adds to the elucidation of the histogenesis of testicular interstitial tumours and may facilitate histopathological diagnosis.

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Section: Discussionmentioning

confidence: 99%

Abundance of DLK1, differential expression of CYP11B1, CYP21A2 and MC2R, and lack of INSL3 distinguish testicular adrenal rest tumours from Leydig cell tumours

Lottrup

Nielsen

Skakkebæk

et al. 2015

European Journal of Endocrinology

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“…Immunostains may also be of assistance; the adrenogenital "tumor" frequently shows significant reactivity for CD56 and synaptophysin and is negative for androgen receptor, with Leydig cell tumor showing an opposite pattern of immunoreactivty. 231,232 Cystic dysplasia This is a rare lesion associated with ipsilateral renal agenesis and may cause a testicular mass in childhood, with a median age of presentation of 3 years, although patients may present at any time from the neonatal period to adult life. 233 These lesions are felt to arise because of failure of development of the normal mesonephric tubules, leading to defective or absent connection of the rete testis with the efferent ductules of the epididymis.…”

Section: Tumor-like Lesions Of the Testismentioning

confidence: 99%

Testicular and paratesticular tumors and tumor-like lesions in the first 2 decades

Ulbright

Young

2014

Seminars in Diagnostic Pathology

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“…Current immunohistochemical summary data predict that 85–95% of LCTs will show immunoreactivity for inhibin, Mart-1, and calretinin [7]. Two small series have shown that <10% of LCTs will show strong immunoreactivity for synaptophysin [8, 9]. …”

Section: Introductionmentioning

confidence: 99%

Metachronous Bilateral Testicular Leydig-Like Tumors Leading to the Diagnosis of Congenital Adrenal Hyperplasia (Adrenogenital Syndrome)

Vukina

Chism

Sharpless

et al. 2015

Case Reports in Pathology

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A 33-year-old male with a history of left testis Leydig cell tumor (LCT), 3-month status after left radical orchiectomy, presented with a rapidly enlarging (0.6 cm to 3.7 cm) right testicular mass. He underwent a right radical orchiectomy, sections interpreted as showing a similar Leydig cell-like oncocytic proliferation, with a differential diagnosis including metachronous bilateral LCT and metachronous bilateral testicular tumors associated with congenital adrenal hyperplasia (a.k.a. “testicular adrenal rest tumors” (TARTs) and “testicular tumors of the adrenogenital syndrome” (TTAGS)). Additional workup demonstrated a markedly elevated serum adrenocorticotropic hormone (ACTH) and elevated adrenal precursor steroid levels. He was diagnosed with congenital adrenal hyperplasia, 3β-hydroxysteroid dehydrogenase deficiency (3BHSD) type, and started on treatment. Metachronous bilateral testicular masses in adults should prompt consideration of adult presentation of CAH. Since all untreated CAH patients are expected to have elevated serum ACTH, formal exclusion of CAH prior to surgical resection of a testicular Leydig-like proliferation could be accomplished by screening for elevated serum ACTH.

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Histopathological and immunophenotypic features of testicular tumour of the adrenogenital syndrome

Abstract: In addition to clinical information, biochemical profile and histopathological findings, our results suggest that immunohistochemistry using a panel of antibodies including CD56, synaptophysin and androgen receptor is helpful in differentiating TTAGS from LCT.

Cited by 44 publications

References 20 publications

Abundance of DLK1, differential expression of CYP11B1, CYP21A2 and MC2R, and lack of INSL3 distinguish testicular adrenal rest tumours from Leydig cell tumours

Abundance of DLK1, differential expression of CYP11B1, CYP21A2 and MC2R, and lack of INSL3 distinguish testicular adrenal rest tumours from Leydig cell tumours

Testicular and paratesticular tumors and tumor-like lesions in the first 2 decades

Metachronous Bilateral Testicular Leydig-Like Tumors Leading to the Diagnosis of Congenital Adrenal Hyperplasia (Adrenogenital Syndrome)

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