Clinical and Pathological Features Associated With the Testicular Tumor of the Adrenogenital Syndrome

Abstract

In our series medical treatment failed in patients with testicular tumor of the adrenogenital syndrome and conservative surgical therapy was possible in select individuals. We identified special histopathological and immunophenotypic features, including synaptophysin staining, which distinguish testicular tumor of the adrenogenital syndrome from Leydig cell tumor.

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

“…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).…”

Bilateral testicular tumor in a patient with adrenogenital syndrome: A conservative approach

“…TTAGS are bilateral in approximately 83% of cases, they are a benign sequel from an endocrine disease, and appear especially in younger patients [6], between 20 to 40 years old [2,6]. In macroscopic exams TTAGS are big multiple masses with "dark green" nodules, unlike LCT which are small, solid and yellow masses [1,2]. A study of TTAGS by Ashely and co [1], has provided useful microscopic characteristics.…”

“…In macroscopic exams TTAGS are big multiple masses with "dark green" nodules, unlike LCT which are small, solid and yellow masses [1,2]. A study of TTAGS by Ashely and co [1], has provided useful microscopic characteristics. In this study, H & E staining revealed extensive fibrosis with adipose metaplasia in 56 to 78% of the cases of TTAGS, unlike LCT, were 0% showed these characteristics.…”

“…Testicular tumors of adrenogenital syndrome (TTAGS) are an entity presented in boys with congenital adrenal hyperplasia (CAH) [1,2]. These tumors have a benign curse, are usually bilateral, localized near the testicular mediastinum [3][4][5].…”

“…A comprehensive clinical assessment is needed to identify TTAGS in patients with CAH. The differential diagnosis with Leydig Cell Tumor (LCT) is the principal objective in the evaluation of these patients, due to the fact that their management is different [1]. Both medical and surgical treatments are available for preventing gonadal dysfunction [1,2].…”