1/35. adrenal rest tumor of the liver: a case report with immunohistochemical investigation of steroidogenesis. A case of adrenal rest tumor arising in the liver of a 62-year-old male with chronic hepatitis type C is reported. The tumor was clinically non-functioning and required distinction from hepatocellular carcinoma. The yellowish-brown tumor measured 25 x 18 x 15 mm and was located in the subcapsular portion of the right hepatic lobe. Histologically, the tumor presented features similar to those of the adrenal cortex and was predominantly composed of pale cells. Electron micrograph revealed lipid droplets and mitochondria with tubulo-vesicular cristae, consistent with the characteristics of steroid-producing cells. Immunohistochemically, the tumor expressed the adrenal 4 binding protein and a number of enzymes involved in the synthesis of adrenocortical steroids. At surgery, the right adrenal gland was present independently from the liver. This hepatic tumor was considered to be an adrenal rest tumor with steroidogenic capability. ( info) |
2/35. Successful treatment with ICSI of infertility caused by azoospermia associated with adrenal rests in the testes: case report. 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) |
3/35. Nonfunctioning adrenal rest tumor of the liver: radiologic appearance. We describe the radiologic features of an adrenal rest tumor of the liver. The adrenal rest tumor appeared on ultrasound as a round, well defined, heterogeneous, solid mass in the posterior aspect of the liver, on angiography as a homogeneous hypervascular mass, and on dynamic CT as a mass containing components of both fat density and soft tissue density and showing early fill-in and early fill-out. Adrenal rest tumors should be included in the lists of hypervascular or fat-containing masses in the liver. ( info) |
4/35. Bilateral testicular adrenal rest tissue in congenital adrenal hyperplasia: US and MR features. We describe magnetic resonance (MR) and ultrasonography (US) features of bilateral testicular adrenal rest tissue in a 20-yr-old man with congenital adrenal hyperplasia (CAH). Scrotal ultrasonology detected bilateral homogeneous hypoechoic lesions with well-defined margins and without evidence of sound attenuation. MR is useful in defining the size of lesions, because the contrast resolution is better than with sonography and allows an accurate definition of the extent of disease. This case suggests that US evaluation should be included in periodical follow-up of patients with CAH, while MR may be used in the case of rapid increase in the size of the testicular mass. ( info) |
| 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) |
6/35. Bilateral testicular adrenal rests after bilateral adrenalectomies in a cushingoid patient with von hippel-lindau disease. We report a case of bilateral testicular masses in a 25-year-old man with von hippel-lindau disease presenting with cushingoid symptoms. His medical history was significant for bilateral adrenalectomies secondary to pheochromocytomas, and he began steroid therapy at that time. After exhaustive endocrinologic, radiographic, and physical examinations, the testicular masses were postulated to be active adrenal rest tissue. Bilateral testicular venous sampling found elevated glucocorticoids that were responsive to dexamethasone suppression, which confirmed the testicular masses as testicular adrenal rests without the need for surgical intervention. Successful conservative management consisted of appropriate steroid manipulation and radiographic evaluation and resulted in the resolution of presenting symptoms, a decrease in size of the bilateral testicular masses, and testicular conservation in this young man. ( info) |
7/35. Testicular adrenal rest tumours in salt wasting congenital adrenal hyperplasia (in vivo and in vitro studies). We describe the case of a 20-year-old patient with salt-wasting congenital adrenal hyperplasia (CAH) related to 21-hydroxylase deficiency. Bilateral craggy testicular tumours were found, requiring histological evaluation. Prior to the surgical procedure, the patient was treated with dexamethasone (he presented cortisol deficiency) and was stimulated with ACTH. High levels of 11beta-OH steroids measured in the gonadal vein, compared with peripheral blood samples suggested the presence of adrenal rests. Incubation of the tumours (which could not be differentiated histologically, from Leydig tissue), with radioactive steroid precursors was carried out. The results revealed the testicular tumours were of adrenal tissue origin, associated with 21-hydroxylase deficiency. The patient's non-compliance to glucocorticoid treatment was the main cause of his hypogonadotropic hypogonadism. ( info) |
8/35. testis sparing surgery for steroid unresponsive testicular tumors of the congenital adrenal hyperplasia. The association between testicular tumors/nodules and congenital adrenal hyperplasia (CAH) has been recognized for many years. Tumors are considered to be an aberrant adrenal tissue that has descended with the testes and has become hyperplastic due to ACTH stimulation. The recommended treatment consists of increasing the glucocorticoid dose to suppress ACTH secretions. If the testicular size is not reduced after suppression therapy or a side effect of glucocorticoid dose is noted, surgical intervention should be considered. We diagnosed steroid unresponsive testicular tumors of the CAH in two patients who were treated by testicular sparing tumor enucleation. We believe that testis sparing surgery is the procedure of choice for all patients with testicular adrenal rest tumor, since it maximizes future fertility potential. ( info) |
9/35. Ovarian adrenal rest tissue in congenital adrenal hyperplasia--a patient report. We report a young girl who died in an Addisonian crisis due to previously undiagnosed congenital adrenal hyperplasia (CAH), in whom ovarian adrenal rest tissue was detected at postmortem histopathological examination. This is a very rare complication in female patients with CAH with only two previously reported cases. ( info) |
10/35. Testicular adrenal rest hyperplasia due to 21-hydroxylase deficiency: a case report. Bilateral testicular tumors are a rare complication of congenital adrenal hyperplasia. It can be extremely difficult to distinguish histologically between Leydig cell tumors and adrenocortical rest hyperplasia, which may lead in some cases to unnecessary orchidectomy. A 7-yr-old boy was admitted because of precocious puberty and enlargement of testicles. Hormonal studies established the diagnosis of congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Testicular biopsy revealed interlacing strands, cords, and rests of cells resembling interstitial (Leydig) cells but with no Reinke crystalloids. Here we report a case of testicular adrenal rest hyperplasia in congenital adrenal hyperplasia and discuss the pathological and clinical features and origin of this rare lesion by using immunohistochemical evaluation. ( info) |