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Query: UMLS:C0027651 (tumor)
 685,946 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Thirty-four cases of corticosurrenaloma with clinical onset before 15 years of age have been studied. Higher frequency in girls (65 percent of the cases) and in young age (80 percent before 5 years of age, 43 percent before 2 years), association with personal or familial other tumors and malformations, are noticeable features. Virilism is the major manifestation in children (83 percent of the cases), either isolated or associated with hypercortisolism and/or feminization. Hormonal assays are of little value, and the dynamic adrenal tests are of some help only in small tumors or isolated hypercortisolism. Radiological diagnosis by urography with cavography, arteriography, is easy in most cases. Evaluation of prognosis is very difficult. Clinical and biological data are not significant, histological data are often of little help. Only a very large mass, and occurrence of metastases (liver, lungs) evidence malignancy. This series does not allow to assess definitely the best therapeutic regimen. Surgery has to be performed as soon as possible. The effects of radiations have not yet been ascertained. High doses of op'DDD have led to a sustained remission of the tumor or metastases in 2 patients, and perhaps to a longer survival in 4 others.
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PMID:[Corticosuprarenaloma in children]. 12 62

Extensive hormonal evaluation was performed in a girl with adrenal carcinoma during the primary tumor stage, following adrenalectomy, during the period when metastases were evident and while on treatment with o,p'-DDD. At the age of 14 months a diagnosis of congenital adrenal hyperplasia was made and treatment with dexamethasone (0.125 to 0.25 mg/day) resulted in a fall-off in growth rate, normal advancement in bone age, decrease in virilization and suppression of 17- ketosteroid excretion which continued until 4 3/12 years of age when virilization increased. At five years of age elevated serum and urinary androgen levels unsuppressible with dexamethasone were noted. Following removal of a large right adrenal carcinoma, serum and urinary hormone levels returned to normal. There months following surgery, liver metastases were documented associated with elevated levels of serum androgens. With o,p'-DDD treatment, serum dehydroepiandrosterone sulfate (DS) and urinary 17-ketosteroid (17-KS) excretion fell rapidly while there was a delay in the fall of free androgens. The persistence of free steroid secretion with decreased formation of DS suggests that the o,p'-DDD may have altered sulfatase activity before causing tumor necrosis and total decrease in steroidogenesis.
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PMID:Virilizing adrenal tumor in a child suppressed with dexamethasone for three years. Effect of o,p'-DDD on serum and urinary androgens. 13 87

In a 64-year-old woman with a virilizing lipid-cell tumor of the left ovary, serum progesterons, androgens, estrogens, and cortisol levels in the peripheral and ovarian veins were measured. Although virilization was the only symptom of hormone production by the tumor in this patient, endocrine studies showed that several steroids were secreted by this neoplasm. Of the steroids measured, androstenedione was the principal secretory product. Pregnenolone, 17-hydroxypregnenolone, 17-hydroxyprogesterone, dehydroepiandrosterone, and testosterone were also secreted, but in quantities which were one third to one sixth the amount of androstenedione. The tumor's pattern of hormone secretion was similar to patterns of steroid production by ovarian stromal cells found in previously reported in vitro studies. This case and a review of the literature demonstrate that androstenedione appears to be the predominant secretory product of lipid cell tumors, whereas testosterone is the predominant secretory product of hilus cell tumors.
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PMID:Androgen, estrogen, and progesterone by a lipid cell tumor of theovary. 14 2

A 2 1/2 year old girl with virilization and advanced bone age was found to have a leftsided adrenocortical adenoma. The secretion of mainly androgens besides estrogens was not suppressible with dexamethasone. Plasma cortisol levels were not elevated but did not show any circadian rhythm. The tumor was localized by retroperitoneal instillation of gas, intravenous urography and tomography. After complete removal of the tumor the originally strongly elevated excretion of 17-ketosteroids sank back to normal, the acceleration of skeletal development slowed down. The right adrenal cortex was not atrophic, 2 1/2 years after the operation the child is well and without evidence of recurrent disease. Differential diagnosis as well as ultrastructor and histology of the tumor are discussed with reference to the literature.
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PMID:[Adrenocortical adenoma in a 2 1/2 year old girl causing virilization and accelerated growth. Clinical and morphological results (author's transl)]. 19 May 31

The tumor of lutein cells of the ovary during pregnancy was first described by Sternberg (1963) as "luteoma of pregnancy". Up to 1973/74 66 cases were published. This benign tumor occurring only during pregnancy probably arises under stimulation of chorionic gonadotropin and may cause a virilization of the mother and female infant. After birth the ovarian tumors regress spontaneouly. Lutein cell tumors are usally discovered incidentally at laparotomy (cesarean section) in late pregnancy and require no radical surgical treatment. The feature of these tumors and some related questions are discussed on the basis of on own observation being, to our knowledge, the first case of this type in Central Europe.
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PMID:[Luteoma of the ovary during pregnancy (author's transl)]. 20 35

The clinical course of a 26-year-old primigravid woman with a virilizing luteoma of pregnancy is described. It was demonstrated by endocrine studies that the tumor secreted an excessive amount of testosterone. After removal of the luteoma in week 32 of pregnancy, the virilizing symptoms of the mother completely regressed. A normal male infant was delivered in week 39. From 100 luteomas of pregnancy described in the literature, 26 cases have been cited to elicit endocrine virilizing activity. An evaluative synopsis indicates that including the case described in this paper only 13 can be regarded as a clinical, morphologic, and functional entity. This entity should be differentiated from the inhomogeneous group of luteomas as the "androluteoma syndrome of pregnancy." It is characterized by the following major criteria: virilization, beginning with the second trimester of pregnancy; histologic criteria of a luteoma of pregnancy; production of testosterone, leading to excessive plasma levels (exceeding the concentration of androstenedione), and masculinization of female fetuses.
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PMID:Androluteoma syndrome of pregnancy. 20 61

The case of a 14-year-old girl with hirsutism and virilism due to the secretion of ectopic ACTH by an adrenal medullary tumor is described. At the age of 5 years changes in appearance had begun with masculinization. The effect of ACTH-like material, measured by radioimmunoassay in plasma and in tumor tissue, was compensated partially by the hypothalamo-pituitary-adrenal feedback mechanism. Increased concentrations of dehydroepiandrosterone, estrone and testosterone in plasma and of 17-ketosteroids and free cortisol in urine originated in the adrenals. After operation of the tumor menarche began spontaneously, hirsutism disappeared and testosterone plasma concentrations returned to normal. An adrenogenital syndrome was excluded.
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PMID:[Virilism due to an adrenal medullary tumor with ectopic ACTH syndrome (author's transl)]. 21 12

The peripheral levels of 17-hydroxypregnenolone (17delta5P), progesterone (P), 17-hydroxyprogesterone (17P), testosterone (T), 5alpha-dihydrotestosterone (DHT), androstenedione (A), androst-5-ene-3beta,17beta-diol (delts5diol), dehydroepiandrosterone (DHEA), dehydroepiandrosterone sulfate (DHEA-S), estradiol-17beta (E2), and cortisol (F) were measured in peripheral and right adrenal venous blood of an amenorrheic patient with a right virilizing adrenal adenoma. Urinary 17-ketosteroids were markedly elevated and were not suppressed on a low dose of dexamethasone (Dex) for 7 days. Peripheral T level was 1.2 ng/ml and DHEA-S was 13,500 ng/ml. Calculations of the ratios of adrenal venous gradients for delta5 and delta4 steroids suggest that the predominant pathway of steroid secretion used by the tumor was as follows: pregnenolone (delta5P) leads to 17delta5P leads to DHEA leads to A leads to T. Following removal of the adenoma, T returned to normal levels but DHEA-S was still above normal at 4100 ng/ml. The patient became eumenorrheic with marked improvement at her hirsutism and virilization.
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PMID:Peripheral and adrenal venous levels of steroids in a patient with virilizing adrenal adenoma. 21 46

The ovarian-peripheral gradients of various delta4 and delta5 steroids were determined for a patient with virilizing arrhenoblastoma. The high peripheral testosterone level accompanying this tumor results from increased precursor supply from both the delta4 and delta5 pathways, with the delta5 pathway predominating, and from negligible aromatase activity. A review of 45 cases of androgen-producing ovarian tumors with measurement of peripheral venous testosterone, and of 24 cases with measurement of ovarian venous testosterone, and a comparison with findings in 159 patients with hirsutism of functional origin reveal the following 1) An androgen-producing tumor must be ruled out when peripheral testosterone exceeds 2 ng/ml; 2) an ovarian venous testosterone level exceeding 20 ng/ml generally accompanies a tumor, particularly when the tumor is less than 5 cm in diameter; and 3) virtually all (98%) of the tumors reviewed were accompanied by virilization, regardless of the peripheral concentration of testosterone.
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PMID:Peripheral and ovarian venous concentrations of various steroid hormones in virilizing ovarian tumors. 21 47

A 35-yr-old woman with Nelson's syndrome presented with amenorrhea and virilization. Serum testosterone (T) concentration was 605 ng/dl and fell to 33 ng/dl when dexamethasone was administered. The MCR of T fell from 1383 to 991 liters/day and the T production rate decreased by 96%. With administration of synthetic ACTH, T concentration rose to 338 ng/dl. Plasma ACTH concentration paralleled T during repeated suppression testing, suggesting that T secretion was dependent on ACTH hypersecretion. Preoperative and intraoperative ovarian vein catheterization suggested that the predominant source of androgen production was from the right ovarian vein. Laporatomy revealed multiple paraovarian tumors in the right mesosalpinyx and mesovarium. Incubation of tumor slices and ovarian tissue with [3H]pregnenolone and [14C]17-hydroxyprogesterone demonstrated conversion of both precursors to T by the tumor and confirmed that the tumors were the source of androgen excess. The microscopic appearance of the tumors closely resembled the morphology of testicular and paratesticular tumors of men with congenital adrenal hyperplasia and Nelson's syndrome. The analogous dependency of the tumors on ACTH hypersecretion in men with paratesticular tumors and in this woman with paraovarian tumors suggests that the tumors may arise in both males and females from a common steroid-secreting cell of adrenogenital origin.
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PMID:Adrenocorticotropin-dependent virilizing paraovarian tumors in Nelson's syndrome. 22 75


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