No abstract available.
Adrenal Rest Tumor* ; Liver*

Both benign and malignant tumors in adrenal glands could make hypersecretion syndrom arised. Benign tumor usually has small size, homogenous intensity on cross-section. Opening surgery to remove the adrenal gland remains a safe and effective operation. However, a well trained surgeons can remove the tumors by endoscopy. It is an invasive interference with a short term hospitalisation with very low rate of complications.
Adrenal Rest Tumor ; Therapeutics ; Neoplasms ; surgery ; Therapeutics

Testicular adrenal rest tumors (TART) occur often as asymptomatic nodules in corticotropin-dependent lesions aberrant adrenal tissue in congenital adrenal hyperplasia (CAH) patients. The present manuscript is about an unusual case of a 16-year-old CAH patient due to 11beta-hydroxylase deficiency. He underwent testicle biopsy because of testicle tumor suspicion and diagnosed with TART.
Adolescent ; Adrenal Hyperplasia, Congenital* ; Adrenal Rest Tumor* ; Biopsy ; Humans ; Testis

Testicular adrenal rest tumors are a well-known complication in male patients with congenital adrenal hyperplasia. Corticosteroid suppressive therapy usually results in the regression of these tumors. We describe a patient with 21-hydroxylase deficiency who developed bilateral testicular masses. Despite steroid suppressive therapy, the tumors did not regress and hormonal control was poor. Consequently, bilateral partial orchiectomies were performed.
Adrenal Hyperplasia, Congenital ; Adrenal Rest Tumor ; Child ; Humans ; Male ; Orchiectomy ; Steroid 21-Hydroxylase ; Testicular Neoplasms

OBJECTIVETo investigate the early diagnosis and treatment of congenital adrenal hyperplasia (CAH) complicated by testicular adrenal rest tumors (TART).

METHODSWe retrospectively analyzed the clinical data of 1 case of late-onset CAH complicated by TART diagnosed and treated in Xiamen Women and Children Health Care Hospital.

RESULTSThe patient was a 15 years old boy, short statured and dark skinned, with skin pigmentation in the gum and external genital, secondary sex characteristics of the adult and irregular tubercles palpable in the bilateral testes. Laboratory examinations showed obviously increased levels of ACTH, 17-KS, DHEA-S and progesterone and evidently decreased levels of FSH, LH and CO. The low-dose dexamethasone suppression test reduced ACTH and DHEA-S to normal. Imaging examinations revealed soft tissue density in the bilateral adrenal glands, especially on the right, and irregularly increased volume of the bilateral testes, particularly on the left, with heterogeneous signals and septas and surrounded by the fluid signals. Histopathological examinations showed the eosinophilic cytoplasm to be polygon- or round-shaped, interstitium-like cells arranged in line, and lipopigment in the endochylema. Immunohistochemical results were negative for testicular interstitial cell tumor. The clinical signs of the patient were improved after 3 months of dexamethasone treatment, the hyperplastic nodules in the left testis decreased obviously and those in the right testis disappeared after 6 months, and the hyperplastic nodules in the adrenal glands vanished after 9 months.

CONCLUSIONBased on the clinical manifestations and the results of auxiliary examinations, this case was diagnosed as late-onset CAH complicated by TART, which was attributed to the continued surge of ACTH induced by corticoadrenal insufficiency. Sufficient dexamethasone treatment could make the TART decrease or disappear and the CAH vanish; it could also improve the clinical symptoms and bring the laboratory results to normal.

Adrenal Rest Tumor ; pathology ; surgery ; Female ; Humans ; Liver Neoplasms ; pathology ; surgery ; Middle Aged

We experienced a boy manifesting sexual precocity with unilateral testicular tumor, who was finally diagnosed as CAH with 21-hydroxylase deficiency. Initial laboratory findings were compatible with peripheral precocious puberty. Ultrasonogram for testes showed heterogenous high echoic mass in the right testicle. Radical orchiectomy was performed and its pathology revealed benign Leydig cell tumor without Reinke crystal. But, testosterone was not decreased after 1 month of surgery. Second laboratory exam revealed increased DHEA-S and 17-hydroxyprogesterone. Finally, this case was recognized as CAH(simple virilizing type) with unilateral testicular adrenal rest tumor. In conclusion, CAH should always be considered during etiologic study for the male sexulal precocity even with unilateral testicular tumor.
17-alpha-Hydroxyprogesterone ; Adrenal Hyperplasia, Congenital ; Adrenal Rest Tumor ; Humans ; Leydig Cell Tumor* ; Male ; Orchiectomy ; Pathology ; Puberty, Precocious ; Steroid 21-Hydroxylase ; Testis ; Testosterone ; Ultrasonography

PURPOSE: In male patients with congenital adrenal hyperplasia (CAH), the presence of testicular adrenal rest tumors (TARTs) have been reported, however their prevalence and clinical manifestations are not well known. Untreated TARTs may lead to testicular structural damage and infertility. This study was conducted to investigate the prevalence of TARTs in male patients with CAH, and characterize the manifestations to identify contributing factors to TART. METHODS: Among 102 CAH patients aged 0-30 years, 24 male patients have been regularly followed up in our outpatient clinic at Severance Children's Hospital from January 2000 to December 2014. In order to reveiw the characteristics of TART patients, we calculated the mean levels of hormones during the 5 years before the time of investigation. Five patients underwent follow-up scrotal ultrasonography (US) after adjusting the dosage of glucocorticoids. RESULTS: TARTs were detected in 8 of the 13 patients (61.5%). The median age of TARTs diagnosis was 20.2 years with the youngest case being 15.5 years old. The mean serum level of adrenocorticotropic hormone (ACTH) was higher in the TARTs patient group compared to the non-TARTs group (P<0.05). The tumor size decreased in 3 cases, slightly increased in 1 case, and had no change in another case. CONCLUSION: The serum ACTH level might be associated with the growth promoting factor for TARTs, but the exact mechanism has not been clearly identified. Screening for TARTs using US is important in male patients with CAH for early-detection and prevention of ongoing complications, such as infertility.
Adrenal Hyperplasia, Congenital* ; Adrenal Rest Tumor* ; Adrenocorticotropic Hormone ; Ambulatory Care Facilities ; Diagnosis ; Follow-Up Studies ; Glucocorticoids ; Humans ; Infertility ; Male* ; Mass Screening ; Prevalence ; Ultrasonography

We experienced two cases of congenital adrenal hyperplasia(CAH), complicated by true precocious puberty with early maturation of the hypothalamic-pituitary-gonadal axis in a boy and a girl. The boy was diagnosed as CAH at 8 years of age, when he showed pseudoprecocity with adrenal rest tumor. The girl was diagnosed at neonate, when she showed ambiguous genitalia and salt-losing symptom. They developed precocious puberty during steroid treatment. Clinically they showed precocity, advanced bone age, and decreased predicted adult height, even though they showed partial gonadotrophin response to luteinizing hormone releasing hormone (LHRH) stimulation test. They were treated with LHRH analogue(leuprolide depot) resulting no further progression of precocity.
Adrenal Hyperplasia, Congenital* ; Adrenal Rest Tumor ; Adult ; Axis, Cervical Vertebra ; Disorders of Sex Development ; Female ; Gonadotropin-Releasing Hormone ; Humans ; Infant, Newborn ; Male ; Puberty, Precocious*

Testicular adrenal rest tumors (TARTs) are considered to be formed from aberrant adrenal tissue that has become hyperplastic because of elevated adrenocorticotropic hormone (ACTH) in male patients with congenital adrenal hyperplasia (CAH). A 6-year-old boy presented with testicular enlargement and pubic hair. He was diagnosed with CAH complicated by precocious puberty. However, he was not followed-up. At the age of 17, he visited the outpatient clinic because of testicular enlargement and short stature. His right and left testicles were 10x6 cm and 7.5x4.5 cm, respectively. His height was 155.1 cm (standard deviation score [SDS], -2.90). The diagnosis of CAH due to 21 hydroxylase deficiency was confirmed by mutation analysis of CYP21A2. Histological examination of the testes showed large, polygonal, eosinophilic cells with round nuclei and prominent nucleoli, which were suggestive of TARTs. He was treated with dexamethasone for 3 weeks and tumors regressed. Subsequently, dexamethasone was replaced by prednisolone and 9alpha-fludrocortisone; thereafter, the reduced testis size has been maintained.
Adrenal Hyperplasia, Congenital ; Adrenal Rest Tumor ; Adrenocorticotropic Hormone ; Ambulatory Care Facilities ; Child ; Dexamethasone ; Eosinophils ; Hair ; Humans ; Male ; Prednisolone ; Puberty, Precocious ; Steroid 21-Hydroxylase ; Testis