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Cases reported "Adrenal Rest Tumor"

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1/16. 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.
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2/16. Nonfunctioning adrenocortical carcinoma.

    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.
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3/16. 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.
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4/16. Ovarian vein sampling in rapidly progressing virilization. A case report.

    A woman presented with progressive hirsutism, deepening of the voice, clitoromegaly and increased libido. The preoperative serum testosterone level was 2,042 ng/dL. The intraoperative ovarian venous blood testosterone levels were 56,327 ng/dL on the left and 1,417 on the right. After a bilateral salpingo-oophorectomy, the serum testosterone level was 20.7 ng/dL. Initial pathologic examination of the ovaries revealed no potential source of testosterone, but reexamination revealed a pure lipoid cell tumor. Intraoperative ovarian vein androgen measurements can be helpful in establishing a histopathologic diagnosis in cases of microscopic virilizing ovarian neoplasms. Ovarian vein sampling for androgen was essential in locating this patient's microscopic tumor.
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5/16. Steroidogenic enzyme activities, morphology, and receptor studies of a testicular adrenal rest in a patient with congenital adrenal hyperplasia.

    Steroid-secreting tumors of the testis have generally been considered to be of Leydig cell origin. Testicular tumors in patients with congenital adrenal hyperplasia have been thought to be adrenal rests, but no conclusive evidence supporting the hypothesis has been presented. We report a morphological and biochemical analysis of a patient with 21-hydroxylase deficiency who developed bilateral nodular hyperplasia of steroid-secreting tissue within the testis, despite suppression therapy with both exogenous glucocorticoids and testosterone. The tissue was formed of confluent nodules of homogenous cells. Electron microscopy showed the cells to have abundant smooth endoplasmic reticulum, well developed golgi apparatus, and mitochondria with predominantly tubular cristae, features characteristic of steroid-secreting cells of adrenocortical origin. Crystals of Reinke were not observed. Functional studies in vivo showed a marked response to ACTH infusion, with 17-hydroxyprogesterone rising from 56 to 13,500 ng/mL, cortisol from less than 2 to 19 micrograms/dL, and testosterone from 369 to 629 ng/dL, with an attendant increase in testicular size and pain over 48 h. Receptor studies in vitro revealed no gonadotropin receptors, but abundant angiotensin-II receptors. Enzyme activity analysis in vitro showed undetectable 21-hydroxylase activity and an enzyme profile consistent with adrenocortical cells rather than leydig cells. Based on these morphological and biochemical findings, we conclude that the nodular steroidogenic tissue that replaced this patient's testes was of adrenal origin. The study documents for the first time the development of adrenocortical tumors from adrenal rest tissue within the testis.
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6/16. Bilateral testicular tumours in congenital adrenal hyperplasia: a continuing diagnostic and therapeutic dilemma.

    Bilateral testicular tumours are a rare but well described complication of congenital adrenal hyperplasia (CAH). It can be extremely difficult to distinguish histologically between Leydig cell tumours and adrenocortical rest hyperplasia which may lead in some cases to unnecessary orchidectomy in young men. A 26-year-old male in whom CAH had been diagnosed neonatally, was referred for further investigation of a craggy mass in the left testis. Six months earlier, right orchidectomy had been performed after presentation with bilateral enlarged, hard testes and azoospermia. The tumour was interpreted to be a malignant interstitial cell tumour of the testis. Our investigations revealed that his CAH was inadequately controlled and that the hormonal secretion from the remaining left testicular mass was ACTH dependent. Percutaneous venous sampling revealed high concentrations of cortisol and other adrenocortical hormones coming from the gonadal vein. dexamethasone suppressed these adrenal hormones and reduced the size and softened the consistency of the testicular mass. Total disappearance of the mass was achieved by using dexamethasone, given in a reverse circadian rhythm regimen. spermatogenesis returned and the patient's wife became pregnant after 6 months; she gave birth to a normal male infant. To our knowledge, this is the first time in this clinical setting that fertility has been achieved. This case highlights the need for both a functional and histological assessment of such 'tumours' in CAH prior to orchidectomy.
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7/16. Lipid cell tumor of the ovary associated with endometrial adenocarcinoma--a case report.

    Presented here is a case of a 54-year-old woman with a lipid cell tumor of the ovary associated with endometrial adenocarcinoma. Lipid cell tumor of the ovary is a rare tumor. From the previous literature, there are only four cases reported to be associated with endometrial adenocarcinoma. The history and the pathological findings will be presented along with the review of the literature.
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8/16. Lipid cell tumor of the ovary: steroid hormone secretory pattern and localization using 75Se-selenomethylcholesterol.

    Ovarian lipid cell tumors are rare and can be difficult to localize by conventional means. We report a postmenopausal patient where rapid progression of symptoms and very high sebum levels suggested the presence of such a tumor which could not be visualized by computed tomography or ultrasound. Localization was accomplished noninvasively using 75Se-selenomethylcholesterol. Peripheral and ovarian vein steroid concentrations suggest that the tumor produced androgens preferentially by way of the delta 4 steroidogenesis pathway.
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9/16. Lipid cell tumor of the ovary in reference to adult-onset congenital adrenal hyperplasia and polycystic ovary syndrome. A case report.

    A 30-year-old woman with a virilizing lipid tumor was initially suspected of having adult-onset congenital adrenal hyperplasia (CAH) when her plasma 17-hydroxyprogesterone (17OHP) concentration was found to be very high (298-3,170 ng/dL), to drop in response to an overnight dexamethasone (dex) suppression test (79 ng/dL) and to rise briskly 15 minutes after ACTH administration (751 ng/dL). However, the effect of dex was not sustained or complete: the pregnanetriol excretion dropped only from 5.5 to 4.4 mg daily. Furthermore, the plasma testosterone was inappropriately high (235-537 ng/dL) for adult-onset CAH and was more responsive to endogenous and exogenous gonadotropin stimulation than to ACTH. In addition, there was no evidence of 11 beta-hydroxylation of 21-deoxycorticoids, as would be expected in CAH. Removal of the tumor completely reversed the virilization and the abnormal responses to ACTH and human chorionic gonadotropin. The contralateral ovary bore lipid-laden stromal cells in the deep paracortex that bore a striking resemblance to the tumor cells, and a cyst in that ovary had fluid with a steroid pattern virtually identical to that of the tumor, with an androstenedione: 17OHP ratio of 5:1. There was not evidence of polycystic ovary disease. The clinical picture of type II polycystic ovary syndrome (PCOS) gradually evolved over a one-year period postoperatively: plasma-free testosterone became mildly elevated and was not dex suppressible. The 17OHP response to ACTH became slightly excessive. However, there was no evidence of tumor on computed tomography.(ABSTRACT TRUNCATED AT 250 WORDS)
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10/16. leydig cell tumor of testis.

    In adult patients with leydig cell tumor of the testis, endocrinologic signs occur in 30 per cent of the cases and often precede the onset of a palpable testicular mass. gynecomastia is the most common endocrinologic manifestation and probably is due to increased estrogen secretion by the leydig cells. In the patient with adrenogenital syndrome and testicular enlargement it is difficult to distinguish leydig cell tumor from adrenal rest hypertrophy. Four patients with leydig cell tumor and endocrinologic manifestations are discussed; three are adults who presented with gynecomastia and the fourth is a patient with congenital adrenogenital syndrome. In the adult patient inguinal orchiectomy is the treatment of choice, while in the patient with adrenogenital syndrome initial management by high-dose steroid suppression should be attempted prior to testicular exploration.
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Last update: April 2009
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