Adrenal Rest Tumor
Neoplasm derived from displaced cells (rest cells) of the primordial ADRENAL GLANDS, generally in patients with CONGENITAL ADRENAL HYPERPLASIA. Adrenal rest tumors have been identified in TESTES; LIVER; and other tissues. They are dependent on ADRENOCORTICOTROPIN for growth and adrenal steroid secretion.
Adrenal Hyperplasia, Congenital
A group of inherited disorders of the ADRENAL GLANDS, caused by enzyme defects in the synthesis of cortisol (HYDROCORTISONE) and/or ALDOSTERONE leading to accumulation of precursors for ANDROGENS. Depending on the hormone imbalance, congenital adrenal hyperplasia can be classified as salt-wasting, hypertensive, virilizing, or feminizing. Defects in STEROID 21-HYDROXYLASE; STEROID 11-BETA-HYDROXYLASE; STEROID 17-ALPHA-HYDROXYLASE; 3-beta-hydroxysteroid dehydrogenase (3-HYDROXYSTEROID DEHYDROGENASES); TESTOSTERONE 5-ALPHA-REDUCTASE; or steroidogenic acute regulatory protein; among others, underlie these disorders.
Successful treatment with ICSI of infertility caused by azoospermia associated with adrenal rests in the testes: case report. (1/13)
Congenital adrenal hyperplasia (CAH) is a well-recognized, but uncommon, cause of azoospermia and infertility in men. Commonly this is due to undertreatment of excessive adrenal androgen secretion which suppresses gonadotrophin stimulation of the testes. A less common complication of CAH is development of adrenal tissue within the testes; this is important to recognize because it may be confused with malignancy leading to unnecessary surgery. In this case report, a man is described with simple virilizing CAH due to 21-hydroxylase deficiency who presented with azoospermia and was found to have adrenal rests. Investigations concluded that there was adequate adrenal suppression with glucocorticoids and that azoospermia was due to obstruction by adrenal rest tissue, strategically situated at the hilum of the testes. Spermatozoa were able to be retrieved by testicular aspiration from the man and these were used to successfully establish a pregnancy using intracytoplasmic sperm injection of his wife's oocytes. (+info)Nonfunctioning adrenocortical carcinoma. (2/13)
The rarity of adrenocortical carcinoma prompted us to report a case who came with a history of swelling in the left flank associated with pain, weakness and loss of appetite. Ultrasonography revealed a left retroperitoneal mass which was removed by radical surgery along with the left kidney and spleen. On histopathological examination, a diagnosis of adrenocortical carcinoma was made. (Hough criteria score 2.97). The cells of the tumor were arranged in closely packed columns and cords supported by fibrovascular stroma. There was no evidence of metastasis. (+info)Prevalence of testicular adrenal rest tumours in male children with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. (3/13)
OBJECTIVE: Testicular adrenal rest tumours (TART) are a well-known complication in adult male patients with congenital adrenal hyperplasia (CAH), with a reported prevalence of up to 94%. In adulthood, the tumours are associated with gonadal dysfunction most probably due to longstanding obstruction of the seminiferous tubules. The aim of our study was to determine the presence of TART and their influence on gonadal function in childhood. DESIGN: Retrospective study. PATIENTS AND METHODS: Scrotal ultrasound was performed in 34 children with CAH due to 21-hydroxylase deficiency who were between 2 and 18 years old. FSH, LH, testosterone and inhibin B concentrations were measured in serum of 27 patients. RESULTS: TART were detected by ultrasound in 8 out of 34 (24%) children. In two of them, bilateral tumours were found. All lesions were located in the rete testis. Seven patients had the salt-wasting type of CAH; one patient had the simple virilising type of CAH. Mean tumour size was 4.1 mm (range 2-8 mm). In none of the patients were the tumours palpable. Two children with TART were between 5 and 10 years old, the other six children were above 10 years old. In all children with TART, LH, FSH, testosterone and inhibin B levels were similar to the patients without TART. CONCLUSION: TART can be found in CAH children before the age of 10 years. The absence of gonadal dysfunction in our group of children suggests that gonadal dysfunction as frequently reported in adult CAH patients with TART develops after childhood. (+info)An adrenal rest tumour in the perirenal region in a patient with congenital adrenal hyperplasia due to congenital 3beta-hydroxysteroid dehydrogenase deficiency. (4/13)
(+info)The prevalence of testicular adrenal rest tumors and associated factors in postpubertal patients with congenital adrenal hyperplasia caused by 21-hydroxylase deficiency. (5/13)
Development of a testicular adrenal rest tumor (TART) is common in males with congenital adrenal hyperplasia, and it can be an important cause of infertility. In the present study, we observed the prevalence of TARTs, and analyzed its associated factors in patients with 21-hydroxylase deficiency. Testicular ultrasonography was performed in 48 postpubertal male patients aged 10.6 to 27.1 years. To determine whether patients were undertreated, we analyzed the serum 17-hydroxyprogesterone (17-OHP) levels to the time of ultrasonographic measurement and calculated the percentage of measurements when serum 17-OHP level was >10 ng/mL relative to the total number of measurements during the follow-up period. We divided the 6-year period before ultrasonographic measurement (time 0) into three 2-year intervals and calculated the average concentration of serum 17-OHP in each interval to give a -2(nd) to 0 year-average concentration (-2-0YAC), -4-2YAC and -6-4YAC. A TART was detected by ultrasonography in 31 of 48 patients (64.6%) and the median maximal cross-sectional area of the TARTs was 0.71 (0.03, 4.95) cm(2). The corrected final adult height was lower, and -4-2YAC and body mass index were higher in patients with TART than in those without. After controlling for the type of 21-hydroxylase deficiency, hydrocortisone-equivalent dose, age, and -6-4YAC, the size of TART was associated with a high undertreatment percentage with a marginal statistical significance. These results suggest that strict disease control is mandatory and regular examination with testicular ultrasonography is recommended in male patients, regardless of the type of 21-hydroxylase deficiency. (+info)Testicular adrenal rest tumor in infertile man with congenital adrenal hyperplasia: case report and literature review. (6/13)
(+info)Fertility, sexuality and testicular adrenal rest tumors in adult males with congenital adrenal hyperplasia. (7/13)
(+info)Testicular adrenal rest tumours in boys with congenital adrenal hyperplasia: case report and literature review. (8/13)
Nodular testicular lesions derived from adrenal tissue (testicular adrenal rest tumours - TART) in boys and men with congenital adrenal hyperplasia (CAH) lead to testicular structure damage, spermatogenesis disorders, and infertility. Hyperplasia of the ectopic adrenal tissue in testes is associated with high levels of the adrenocorticotropic hormone (ACTH) in blood serum. The development of non-invasive methods of diagnostic imaging allows detection of testicular lesions in adolescents and children. The basic method for TART detection is imaging with ultrasonography (USG) being the most widely available method. Since these mild testicular lesions can cause impaired fertility, periodic palpation and testicular ultrasonography should be performed in patients with CAH in order to prevent infertility. (+info)An Adrenal Rest Tumor is a rare condition characterized by the presence of ectopic adrenal tissue, usually found in the ovaries or testes, which can produce hormones leading to endocrine disorders such as Cushing's syndrome or Conn's syndrome.
An Adrenal Rest Tumor is a rare, benign (non-cancerous) growth that occurs in the adrenal glands. These tumors are made up of cells called "adrenal rests," which are small clusters of adrenal tissue that can be found outside of the adrenal glands.
Adrenal rest tumors are typically asymptomatic and are often discovered incidentally during imaging studies performed for other medical reasons. However, in some cases, these tumors may produce hormones such as cortisol or aldosterone, leading to symptoms associated with hormonal imbalances, such as Cushing's syndrome or Conn's syndrome.
Treatment for adrenal rest tumors typically involves surgical removal of the tumor. In cases where the tumor is producing hormones, medication may be used to manage the hormonal imbalance before and after surgery. It is important to monitor patients with adrenal rest tumors for recurrence, as these tumors can grow back over time.
Congenital Adrenal Hyperplasia (CAH) is a group of inherited genetic disorders affecting the adrenal glands' functioning, primarily caused by enzyme deficiencies leading to hormonal imbalances, with 21-hydroxylase deficiency being the most common type, resulting in various symptoms such as ambiguous genitalia, precocious puberty, and sexual development disorders, depending on the severity of the enzyme deficiency.
Congenital Adrenal Hyperplasia (CAH) is a group of inherited genetic disorders that affect the adrenal glands, which are triangular-shaped glands located on top of the kidneys. The adrenal glands are responsible for producing several essential hormones, including cortisol, aldosterone, and androgens.
CAH is caused by mutations in genes that code for enzymes involved in the synthesis of these hormones. The most common form of CAH is 21-hydroxylase deficiency, which affects approximately 90% to 95% of all cases. Other less common forms of CAH include 11-beta-hydroxylase deficiency and 3-beta-hydroxysteroid dehydrogenase deficiency.
The severity of the disorder can vary widely, depending on the degree of enzyme deficiency. In severe cases, the lack of cortisol production can lead to life-threatening salt wasting and electrolyte imbalances in newborns. The excess androgens produced due to the enzyme deficiency can also cause virilization, or masculinization, of female fetuses, leading to ambiguous genitalia at birth.
In milder forms of CAH, symptoms may not appear until later in childhood or even adulthood. These may include early puberty, rapid growth followed by premature fusion of the growth plates and short stature, acne, excessive hair growth, irregular menstrual periods, and infertility.
Treatment for CAH typically involves replacing the missing hormones with medications such as hydrocortisone, fludrocortisone, and/or sex hormones. Regular monitoring of hormone levels and careful management of medication doses is essential to prevent complications such as adrenal crisis, growth suppression, and osteoporosis.
In severe cases of CAH, early diagnosis and treatment can help prevent or minimize the risk of serious health problems and improve quality of life. Genetic counseling may also be recommended for affected individuals and their families to discuss the risks of passing on the disorder to future generations.
Testicular neoplasms are abnormal growths or tumors within the testicle, which can be benign (noncancerous) or malignant (cancerous), potentially leading to serious health consequences if not identified and treated promptly.
Testicular neoplasms are abnormal growths or tumors in the testicle that can be benign (non-cancerous) or malignant (cancerous). They are a type of genitourinary cancer, which affects the reproductive and urinary systems. Testicular neoplasms can occur in men of any age but are most commonly found in young adults between the ages of 15 and 40.
Testicular neoplasms can be classified into two main categories: germ cell tumors and non-germ cell tumors. Germ cell tumors, which arise from the cells that give rise to sperm, are further divided into seminomas and non-seminomas. Seminomas are typically slow-growing and have a good prognosis, while non-seminomas tend to grow more quickly and can spread to other parts of the body.
Non-germ cell tumors are less common than germ cell tumors and include Leydig cell tumors, Sertoli cell tumors, and lymphomas. These tumors can have a variety of clinical behaviors, ranging from benign to malignant.
Testicular neoplasms often present as a painless mass or swelling in the testicle. Other symptoms may include a feeling of heaviness or discomfort in the scrotum, a dull ache in the lower abdomen or groin, and breast enlargement (gynecomastia).
Diagnosis typically involves a physical examination, imaging studies such as ultrasound or CT scan, and blood tests to detect tumor markers. Treatment options depend on the type and stage of the neoplasm but may include surgery, radiation therapy, chemotherapy, or a combination of these modalities. Regular self-examinations of the testicles are recommended for early detection and improved outcomes.
Adrenal tumor - Wikipedia
Only 10% of adrenal pheochromocytomas are malignant, while the rest are benign tumors. The most clinically important feature of ... An adrenal tumor or adrenal mass is any benign or malignant neoplasms of the adrenal gland, several of which are notable for ... with surgery employed to remove the tumor once the patient is medically stable. An adrenal incidentaloma is an adrenal tumor ... Adrenal cancer is the presence of malignant adrenal tumors, and includes neuroblastoma, adrenocortical carcinoma and some ...
Emily C. Daugherty, MD
Adrenal Cancer. Adrenal Cortex Cancers. Adrenal Gland Cancers. Adrenal Rest Tumor. Adrenal Tumors ... Adrenal SBRT: a multi-institutional review of treatment outcomes and toxicity. Clinical & experimental metastasis, , ... Surgical and radiation treatment of a paravertebral malignant solitary fibrous tumor: a case report and literature review. AME ...
C-17 Hydroxylase Deficiency: Practice Essentials, Pathophysiology, Etiology
The rare variant of congenital adrenal hyperplasia (CAH) known as 17-hydroxylase deficiency was first described in the 1960s in ... Molecular Characterization of Testicular Adrenal Rest Tumors in Congenital Adrenal Hyperplasia; Lesions with both ... Sexuality and Testicular Adrenal Rest Tumors in Adult Males with Congenital Adrenal Hyperplasia. Eur J Endocrinol. 2011 Dec 9. ... adrenal tumors, CAH due to 11-hydroxylase deficiency, and primary cortisol resistance. [8, 9] ...
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Secondary Hypertension - Harvard Health
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Clinical Applications and the Roles of Transporters in Disposition, Tumor Targeting, and Tissue Toxicity of meta...
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Successful treatment in mice for severe childhood cancer | University of Gothenburg
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O Testicular adrenal rest tumor,O Testicular atrophy,O Testicular dysgenesis,O Testicular fibrosis,O Testicular gonadoblastoma, ... O Adrenal calcification,O Adrenal gland agenesis,O Adrenal gland dysgenesis,O Adrenal hyperplasia,O Adrenal hypoplasia,O ... Adrenal insufficiency,O Adrenal medullary hypoplasia,O Adrenal overactivity,O Adrenal pheochromocytoma,O Adrenocortical ... O Neoplasm of the adrenal cortex,O Neoplasm of the adrenal gland,O Neoplasm of the adrenal medulla,O Neoplasm of the anterior ...
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Genotype of congenital adrenal hyperplasia patients with testicular adrenal rest tumor. European journal of medical genetics ... Giant bilateral adrenal myelolipomas in two Chinese families with congenital adrenal hyperplasia. Endocrine connections 2018 10 ... Mutations in CYP11B1 and congenital adrenal hyperplasia in Moroccan Jews. The Journal of clinical endocrinology and metabolism ... Allele-specific PCR and Next-generation sequencing based genetic screening for Congenital Adrenal Hyperplasia in India. ...
Congenital Adrenal Hyperplasia Caused by 21-Hydroxylase Deficiency - Pediatrics - Merck Manuals Professional Edition
Congenital Adrenal Hyperplasia Caused by 21-Hydroxylase Deficiency - Etiology, pathophysiology, symptoms, signs, diagnosis & ... but others may develop testicular adrenal rest tumors (benign intratesticular masses composed of adrenal tissue that ... of all cases of congenital adrenal hyperplasia Overview of Congenital Adrenal Hyperplasia Congenital adrenal hyperplasia is a ... There... read more (1 General references 21-Hydroxylase (CYP21A2) deficiency causes defective conversion of adrenal precursors ...
Kosuke Oka - Research output - Okayama University
Adult hypogonadism due to testicular adrenal rest tumors. Yamamoto, K., Oka, K., Hasegawa, K. & Otsuka, F., 2021, In: Internal ... An adrenal incidentaloma caused by synchronous and isolated metastasis. Yamamoto, K., Oka, K., Honda, H., Hasegawa, K., ... Neck schwannoma mimicking a thyroid tumor. Oka, K., Iwamuro, M. & Otsuka, F., Dec 2017, In: Journal of General and Family ...
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Surgery for Adrenal Tumors | NYU Langone Health
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hrp0097p2-140 , Adrenals and HPA Axis , ESPE2023. Testicular Adrenal Rest Tumors (TARTs) as presenting symptom of CAH due to ... Background: TARTs mostly occur in congenital adrenal hyperplasia due to 21-hydroxylase deficiency, but were described in other ... and neuroendocrine tumors in 30 40%. Autoimmunity and paraneoplastic syndrome have been proposed as possible pathophysiological ...
Thyroid, Parathyroid, Adrenal, Endocrine Surgery, What is a GHRH-Secreting Tumour (GHRHoma)?
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congenital adrenal hyperplasia - BJUI
Ovarian adrenal rest tumour in a patient with chronically untreated congenital adrenal hyperplasia (CAH). July 27, 2011. /by ... Ovarian adrenal rest tumor (masculinovoblastoma) stimulating adrenal virilism: report of a case with hormone studies. Am J ... 6. Tiosano D, Vlodavsky E, Filmar S, Weiner Z, Goldsher D, Bar-Shalom R. Ovarian adrenal rest tumor in a congenital adrenal ... Abbreviations: CAH - congenital adrenal hyperplasia, OART - ovarian adrenal rest tumour, CT - computer tomography, MRI - ...
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