Cases reported "Adrenal Rest Tumor"

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1/7. 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.
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2/7. 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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keywords = androgen
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3/7. 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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keywords = androgen
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4/7. Lipoid cell tumor of the ovary.

    An androgen-secreting tumor of the left ovary was preoperatively localized in a 55-year-old virilized woman by radioimmunoassay of blood androgens. The blood sample was obtained by bilateral catheterization of the ovarian and adrenal veins using a percutaneous retrograde femoral approach. A total abdominal hysterectomy and bilateral salpingo-oophorectomy were performed, and revealed a lipoid cell tumor of the left ovary and right ovarian stromal hyperplasia. Despite early postoperative return of plasma adrogens to the normal range, virilizing signs persisted almost unchanged 1 year after surgery.
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keywords = androgen
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5/7. Preoperative localization of virilizing tumors by selective venous sampling.

    Two postmenopausal patients with virilization had preoperative localization of ovarian tumors by selective blood sampling from both ovarian and adrenal veins and assay of hormone levels. In the first patient, the peripheral concentrations of testosterone (T), androstenedione, and estrone were 936, 1,508 and 73 pg. per milliliter, respectively, levels which are above the ranges found in normal postmenopausal women. The catheterization study showed an increase in the left ovarian vein of all hormones except cortisol. It was predicted that a tumor was present in the left ovary. At operation a 7 by 4 mm. lipid cell tumour was found. In the peripheral blood of the second patient, the T level (4,518 pg. per milliliter) was markedly elevated and the estradiol concentration (73 pg. per milliliter) was increased. At retrograde catheterization the concentration of T in the right ovarian vein was markedly elevated at 120,400 pg. per milliliter. At operation a hilus cell tumor of the right ovary was found. These two cases represent the third and fourth consecutive androgen-secreting tumors from this institution that have been localized by selective ovarian and adrenal vein catheterization and sampling.
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6/7. hyperandrogenism due to 3 beta-hydroxysteroid dehydrogenase deficiency with accessory adrenocortical tissue: a hormonal and metabolic evaluation.

    1. Adrenal ectopic tissue has been detected in the paragonadal region of normal women. In patients with congenital adrenal hyperplasia due to 21-hydroxylase (21-OH) deficiency, the manifestation of hyperplasia of paragonadal accessory adrenal tissue has been usually reported to occur in males. Probably, this is the first report of a female with 3 beta-hydroxysteroid dehydrogenase (3 beta-HSD) deficiency with ectopic adrenal tissue in ovaries. However, the occurrence of hyperplasia of adrenal rests among women with classical congenital adrenal hyperplasia may not be rare, especially among patients with a late diagnosis. 2. We report a woman with 3 beta-HSD deficiency whose definitive diagnosis was made late at 41 years of age immediately before surgery for the removal of a uterine myoma. During surgery, exploration of the abdominal cavity revealed the presence of bilateral accessory adrenal tissue in the ovaries and in the para-aortic region. The patient had extremely high levels of ACTH (137 pmol/l), DHEA (901.0 nmol/l), DHEA-S (55.9 mumol/l), androstenedione (70.2 nmol/l), testosterone (23.0 nmol/l) and 17 alpha-hydroxypregnenolone (234.4 nmol/l) suggesting 3 beta-HSD deficiency. 3. In view of these elevated androgen levels, with an absolute predominance of DHEA and DHEA-S, we evaluated the effect of this hormonal profile on carbohydrate tolerance and insulin response to glucose ingestion. 4. The patient presented normal glucose tolerance but her insulin response was lower than that of 14 normal women (area under the curve, 3 beta-HSD = 17,680 vs 50,034 pmol/l for the control group over a period of 3 h after glucose ingestion).(ABSTRACT TRUNCATED AT 250 WORDS)
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keywords = androgen
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7/7. Congenital adrenal hyperplasia with testicular tumors, aggression, and gonadal failure.

    Bilateral testicular tumors (adrenal rests) may occur in untreated or poorly controlled congenital adrenal hyperplasia. This case report describes two unique associated phenomena: (1) psychologic disturbances similar to those seen with exogenous androgen abuse, which resolved with appropriate glucocorticoid suppression of androgen over-production by this abnormal adrenal/adrenal rest tissue; and (2) testicular failure which showed a partial, delayed recovery with corticosteroid therapy. The need for a careful history and biochemical screening for all patients with bilateral testicular tumors is reinforced.
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keywords = androgen
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