ABSTRACT
Testicular adrenal rest tumors and adrenogenital syndrome (AGS) - Do not mix up with malignant testicular tumors! Testicular adrenal residual tumors (TARTs) frequently occur in men with adrenogenital syndrome. Without knowledge of AGS, diagnosis is problematic due to difficult differentiation from other testicular tumors. However, early treatment is crucial for maintaining or regaining fertility, among other aspects. This article provides background knowledge for general practitioners.
Subject(s)
Adrenal Gland Neoplasms , Adrenal Rest Tumor , Adrenogenital Syndrome , Testicular Neoplasms , Male , Humans , Adrenal Rest Tumor/diagnosis , Adrenogenital Syndrome/diagnosis , Adrenogenital Syndrome/therapy , Testicular Neoplasms/diagnosis , Testicular Neoplasms/therapy , FertilitySubject(s)
Adrenogenital Syndrome/diagnosis , Adrenogenital Syndrome/therapy , Feminization/prevention & control , Glucocorticoids/administration & dosage , Hormone Replacement Therapy/methods , Virilism/prevention & control , Diagnosis, Differential , Evidence-Based Medicine , Female , Feminization/diagnosis , Humans , Male , Treatment Outcome , Virilism/diagnosisABSTRACT
Testicular tumor of adrenogenital syndrome is a rare and benign anomaly usually presenting as bilateral testicular masses. It is the most important cause of infertility in adult male congenital adrenal hyperplasia. Distinction between testicular tumors of adrenogenital syndrome and Leydig cell tumors can be problematic; it is based on clinical, histopathologic, immunohistochemical and endocrine features. Biopsy is advised in cases of longstanding tumors in infertile patients and when surgery is indicated. Fertility preservation is a key management goal in testicular tumor of adrenogenital syndrome. In stages 2 and 3, intensified glucocorticoid treatment is recommended as a first step treatment. Sparing surgical approach is preferred for tumors of stage 4 and steroid unresponsive masses. Magnetic resonance imaging is recommended before surgery. The only indication of surgery in stage 5 is testicular pain.
Subject(s)
Adrenogenital Syndrome/physiopathology , Adrenogenital Syndrome/therapy , Testicular Neoplasms/physiopathology , Testicular Neoplasms/therapy , Adrenal Rest Tumor/diagnosis , Adrenal Rest Tumor/pathology , Adrenal Rest Tumor/physiopathology , Adrenal Rest Tumor/therapy , Adrenocorticotropic Hormone/blood , Adrenogenital Syndrome/diagnosis , Adrenogenital Syndrome/pathology , Adult , Diagnosis, Differential , Glucocorticoids/therapeutic use , Humans , Leydig Cell Tumor/diagnosis , Leydig Cell Tumor/pathology , Leydig Cell Tumor/physiopathology , Leydig Cell Tumor/therapy , Magnetic Resonance Imaging , Male , Testicular Neoplasms/diagnosis , Testicular Neoplasms/pathology , Testis/pathology , Testis/physiopathologySubject(s)
Adrenogenital Syndrome , Gonadal Disorders , Adrenogenital Syndrome/therapy , Female , Gonadal Disorders/therapy , Humans , Internal Medicine , MaleABSTRACT
Information about the treatment of males with congenital adrenal hyperplasia (CAH) is scarce and there are no therapeutical guidelines. The aim of this review is to provide a survey of the current data. An extensive literature research was performed in PubMed for relevant articles published in the last ten years. The aim in the treatment of adult male CAH patients is preservation of fertility, prevention of an addisonian crisis, blood pressure management, prevention of testicular adrenal rest tumors (TART), maintaining well-being and good quality of life, satisfactory sexual function and prevention of long-term side effects of gluco- and mineralocorticoid therapy. The change from paediatric to adult medicine should be handled in a transition outpatient clinic organized by paediatric and adult endocrinologists. Most studies have included only small numbers of patients. The steroid therapy is usually orientated on an individual basis; but, general guidelines are lacking. It is reported that fertility is often impaired and related to the occurrence of TART. Some of these tumors are responsive to altered glucocorticoid therapy. However, glucocorticoid-resistant TART have been described, and testis-sparing surgery seems to be a treatment option. A future system of regular follow-up visits and standardized therapy guidelines are essential to provide a better medical care and a higher quality of life for male patients with CAH.
Subject(s)
Adrenal Hyperplasia, Congenital/therapy , Adrenogenital Syndrome/therapy , Glucocorticoids/therapeutic use , Mineralocorticoids/therapeutic use , Steroid 21-Hydroxylase/metabolism , Addison Disease/etiology , Addison Disease/prevention & control , Adrenal Hyperplasia, Congenital/complications , Adrenal Hyperplasia, Congenital/etiology , Adrenal Medulla/physiopathology , Adrenal Rest Tumor/etiology , Adrenal Rest Tumor/prevention & control , Adrenogenital Syndrome/complications , Adrenogenital Syndrome/etiology , Adult , Continuity of Patient Care , Humans , Hypertension/etiology , Hypertension/prevention & control , Infertility, Male/etiology , Infertility, Male/prevention & control , Male , Testicular Neoplasms/etiology , Testicular Neoplasms/prevention & controlABSTRACT
OBJECTIVE: To discuss the treatment of male adrenogenital syndrome. METHODS: The clinical data of 17 patients with male adrenogenital syndrome, including 15 cases of congenital adrenal hyperplasia (CAH) and 2 cases of adrenocortical tumors, were analyzed retrospectively. The patients with 21-hydroxylase deficiency (21-OHD) and 11beta-hydroxylase deficiency (11beta-OHD) were treated with adrenocortical hormone, those with 17-hydroxylase deficiency (17-OHD) received sexual glands excision and estrogen besides adrenocortical hormone, and those with adrenocortical tumors underwent surgical removal. RESULTS: Sexual precocity symptoms disappeared and abnormal laboratory results returned to normal in 5 of the 21-OHD patients, who adhered to hormone treatment, and height growth was improved in the other 2, who received the medicine at an early age. The testicular adrenal rest (TAR) tumor was reduced dramatically in 1 case of 21-OHD after treatment. A left TAR found in another 21-OHD patient who discontinued the hormone therapy became softened after the resumption. Sperm could be seen in the semen of 3 21-OHD patients, but in small quantity and of poor quality. One 11beta-OHD patient with sexual precocity symptoms and hypertension became normal after the hormone treatment, and six 17-OHD patients maintained their female sexuality after the hormone treatment and operation. No relapse was found after resection of the adrenocortical tumors. CONCLUSION: Adrenocortical hormone therapy helps improve the height growth and testicular function of CAH patients, and surgical removal is necessary for adrenocortical tumors.
