Testicular Adrenal Rest Tumor, What is Testicular adrenal residual tumor?
Testicular Adrenal Rest Tumor, Testicular adrenal residual tumor (TART); They are non-cancerous masses that develop in the testicles due to congenital adrenal hyperplasia. Testicular masses called adrenal rest tumors develop in 8% of patients with congenital adrenal hyperplasia. It has five stages, its treatment is planned according to the stage. Testicular adrenal rest tumor (TART) is the most important cause of infertility in adult men with congenital adrenal hyperplasia.
Testicular Adrenal Rest Tumor, Testicular Adrenal Residual Tumor (TART) Causes?
Congenital adrenal hyperplasia (CAH) is a group of adrenal steroid synthesis disorders that occur with the deficiency of one of the enzymes required in cortisol synthesis. The most common (more than 95%) type of CAD is 21-hydroxylase deficiency. The second most common type, 11-beta-hydroxylase enzyme deficiency, accounts for 5-8% of all CAD cases. The disease is autosomal recessive inherited. There are two forms of the disease, classical and non-classical. In the non-classical form, hypertension is not observed, but there are signs of hyperandrogenism. 8% of patients with 21-hydroxylase deficiency present with bilateral testicular masses associated with increased adrenocorticotropic hormones, these masses are adrenal rest tumor (TART). The incidence of testicular mass in 11-beta-hydroxylase deficiency is unknown. The complaint of testicular mass is usually seen in the second decade of life. Surgical removal of the testis can be applied according to the symptoms in patients who do not respond to cortisone.
Testicular Adrenal Rest Tumor, Diagnosis of Testicular Adrenal Residual Tumor (TART)
The hypoechoic appearance observed in the testicular mediastinum and around the rete testis in ultrasonographic (USG) examination is helpful in the diagnosis. The appearance of the mass is nodular or lobulated dark brown with transverse fibrous septum. Lesions are usually located in the hilar region of the testis and extend towards the parenchyma, but no lesions extending from the epididymis and spermatic cord to the tunica albuginea. are in the form of dual structures. TART testicular masses are usually isointense on T1 sequence, hypointense on T2 sequence, and show intense contrast enhancement pattern after contrast agent injection. However, all these USG and MRI findings cannot differentiate from testicular cancers without appropriate clinical and endocrine profile.
Testicular Adrenal Rest Tumor, Testicular adrenal residual tumor (TART) Staging
Testicular adrenal rest tumors (TART) are classified into 5 stages according to clinical and histological findings. The response of testicular masses to drug therapy depends on the histological and clinical stage of TART.
Claahsen-van der Grinten et al. adrenal rest tumors-TART based on histological appearance and surrounding testicular parenchyma and clinical observations. Their staging is as follows:
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Stage 1: It is the presence of adrenal residual cells in the rete testis that cannot be detected by scrotal USG.
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Stage 2: Proliferation of adrenal remnant cells in patients with congenital adrenal hyperplasia with increased levels of growth factors such as ACTH k
and growth is observed. At this stage, one or more small hypoechoic lesions are observed on USG.
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Stage 3: Adrenal remnant cell growth suppresses the rete testis, resulting in oligo or azoospermia due to seminiferous tubule obstruction in patients with pubertal or postpubertal congenital adrenal hyperplasia. Increased FSH, LH, and decreased inhibin B levels can be observed. At this stage, high-dose glucocorticoid therapy may reduce tumor volume. However, with discontinuation or reduction of glucocorticoid therapy, re-exacerbation of the tumor can be observed, this treatment is a temporary solution.
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Stage 4: Progressive obstruction in the rete testis may cause fibrosis and focal lymphocytic infiltration in the tumor. At this stage, glucocorticoid therapy is not effective due to fibrosis in the tumor tissue or decrease in ACTH and desensitization. Peritubular fibrosis in testicular tissue is a sign of early testicular damage.
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Stage 5: Chronic obstruction causes deterioration in testicular parenchyma. It causes irreversible testicular damage. Typically, they appear as hypoechoic lesions with bilateral multifocal acoustic shadowing on USG.
Congenital Difficulty in differential diagnosis between testicular masses due to adrenal hyperplasia and other testicular cancers, especially Leydig cell testicular tumor, is an important clinical problem. Testicular masses due to congenital adrenal hyperplasia (TART) are usually detected incidentally. In order to facilitate the differentiation of these two conditions, some features have been defined: investments are made considering whether they are bilateral or not, age, gynecomastia and laboratory findings. The incidence of bilateral Leydig cell tumors has been reported as 3%, whereas 2/3 of TARTs are bilateral, and scrotal masses are usually seen in young adults. Leydig cell testicular tumor is usually observed in children older than 4 years and adults aged 20-50 years. While gynecomastia is observed in 1/3 of patients with Leydig cell tumors, this condition is rarely observed in patients with TART. There are no biochemical markers that would provide a clear distinction between the two conditions. Hydrocortisone production and steroid hydroxylation due to 21-hydroxylase activity in adrenocortical cells can also be observed in Leydig cell tumors.
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