17-ketosteroids and Adrenal-Cortex-Neoplasms

A 53-year-old female had clinical and laboratory findings suggestive of Cushing's syndrome. In contrast to the Cushing's syndrome caused by cortical adenoma, a high level of urinary 17-ketosteroids (17-KS) was also noted. Imaging studies revealed a right adrenal tumor. Right adrenectomy was performed; the surgical specimen revealed a black adenoma consisting of compact cells with numerous pigments which seemed to be lipofuscin in nature. The present case indicates that black adenoma as well as adrenocortical carcinoma should be suspected, when patients with Cushing's syndrome show an increased level of urinary 17-KS excretion.

Topics: 17-Ketosteroids; Adrenal Cortex Neoplasms; Adrenocortical Adenoma; Cushing Syndrome; Female; Humans; Middle Aged

Topics: 11-Hydroxycorticosteroids; 17-Hydroxycorticosteroids; 17-Ketosteroids; Adenoma; Adrenal Cortex Neoplasms; Adult; Female; Humans; Hydrocortisone; Testosterone

We have experienced two male cases of an adrenocortical carcinoma that have produced steroid hormones without endocrinological symptoms. Both men were admitted to hospital because of an abdominal pain and left hypochondral tumors. In each case, an abdominal CT, an ultrasonogram and an angiogram demonstrated a left adrenal tumor, and levels of blood dehydroepiandrosterone sulfate, etiocholanolone, pregnenolone sulfate and urine 17 KS were elevated. The tumors were resected and the histology revealed that they were adrenocortical carcinomas. Postoperatively, the levels of their urine hormones returned to normal values, though in one man recurrence occurred a year later. Only 8 cases of an adrenocortical carcinoma that has produced steroids without specific endocrinological symptoms have been reported in Japan, and we also report on two cases herein.

Topics: 17-Ketosteroids; Adrenal Cortex Hormones; Adrenal Cortex Neoplasms; Adult; Carcinoma; Dehydroepiandrosterone; Dehydroepiandrosterone Sulfate; Etiocholanolone; Humans; Male; Pregnenolone

Topics: 17-Ketosteroids; Adenoma; Adrenal Cortex Neoplasms; Adrenocorticotropic Hormone; Adult; Androgens; Carcinoma; Child, Preschool; Cushing Syndrome; Estrogens; Female; Feminization; Glucocorticoids; Granulosa Cell Tumor; Humans; Leydig Cell Tumor; Male; Mesenchymoma; Middle Aged; Mitotane; Ovarian Neoplasms; Paraneoplastic Endocrine Syndromes; Pregnancy; Progesterone; Prognosis; Sertoli Cell Tumor; Steroids; Testicular Neoplasms; Thecoma; Virilism

Eight cases of adrenocortical tumor are presented with a review of the literature. Although such tumors are rare, they are important causes of inappropriate virilization and Cushing's syndrome in childhood. Clinical virilization with or without hypercortisolism was found in all eight children, who were 5 years old or younger. Excessive linear growth was noted, despite evidence of hypercortisolism. Serum levels of dehydroepiandrosterone, dehydroepiandrosterone sulfate, testosterone, and cortisol were elevated in all cases tested and appear to be useful diagnostic alternatives to the more traditional determinations of urine 17-ketosteroids and 17-hydroxycorticosteroids. Abdominal sonography and computed tomography have proven to be reliable tools for tumor localization. Surgical resection was the definitive therapy in all patients, and perioperative steroid replacement was essential. Histologic diagnosis appeared to have little bearing on prognosis, and the majority of pediatric patients have had clinically benign disease. At a mean follow-up of 3 years, seven of the eight children were alive and had no evidence of tumor recurrence.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adenocarcinoma; Adenoma; Adrenal Cortex Neoplasms; Child, Preschool; Cushing Syndrome; Dehydroepiandrosterone; Female; Follow-Up Studies; Humans; Hydrocortisone; Infant; Male; Puberty, Precocious; Testosterone; Virilism

A patient with adrenocortical carcinoma presented with fever, leukocytosis, and increased acute phase reactants. The tumor was infiltrated with neutrophils. Immunohistochemical staining of the tumor showed positive signal for epithelial neutrophil-activating protein-78, an angiogenic and chemotactic CXC chemokine. Conditioned medium from tumor-derived cells (RL-251) showed high concentration of IL-8, epithelial neutrophil-activating protein-78, Gro alpha, and Gro gamma, angiogenic CXC chemokines with a potential role in tumorigenesis. An adrenal cancer/severe combined immunodeficiency mouse chimera was developed. Mice grew tumors rapidly, and circulating levels of IL-8 and epithelial neutrophil-activating protein-78 were detected. In contrast, animals transplanted with NCI-H295 cells, a nonchemokine-secreting cell line, grew tumors more slowly and did not have detectable chemokine levels. Similar to the patient, mice with RL-251 tumors developed marked leukocytosis and neutrophilia, and their tumors were infiltrated with neutrophils. Mice were passively immunized with epithelial neutrophil-activating protein-78 antisera. A marked decrease in tumor growth was observed. Potential for chemokine production by other adrenocortical tumors was investigated by RT-PCR in archival material. Six of seven adrenal carcinomas and one of three adenomas had cDNA for IL-8; six of seven carcinomas and the three adenomas had cDNA for epithelial neutrophil-activating protein-78. We concluded that the clinical presentation of this case resulted from increased tumor production of chemotactic chemokines. Through their angiogenic and chemotactic properties these chemokines may play an important role in adrenal tumorigenesis.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adenoma; Adrenal Cortex Neoplasms; Adrenocorticotropic Hormone; Aged; Chemokine CXCL5; Chemokines, CXC; Circadian Rhythm; Fever; Humans; Hydrocortisone; Immunohistochemistry; Interleukin-8; Leukocytosis; Male; Neutrophil Activation; Neutrophils; Reverse Transcriptase Polymerase Chain Reaction; Syndrome; Tumor Cells, Cultured

An adrenocortical adenoma causing Cushing's syndrome (Cushing's adenoma) produces a unilateral concordant visualization (UCV) imaging pattern in which the adenoma is only visualized on radioiodocholesterol adrenocortical scintigraphy. But because this imaging pattern is also noted in some patients with adrenal incidentalomas, we examined whether the UCV-incidentaloma was essentially identical with Cushing's adenoma and would develop Cushing's syndrome. The subjects were 9 patients with UCV-incidentalomas (mean size, 30 mm; range, 20-45 mm) and 6 patients with Cushing's adenomas (mean size, 28 mm; range, 25-35 mm). Endocrinological evaluations showed several abnormalities including blunted diurnal rhythm of plasma cortisol within the normal range, low plasma ACTH and/or high 24-hr urinary 17-OHCS levels in 8 of 9 patients with UCV-incidentalomas, but these abnormalities did not meet the diagnostic criteria of Cushing's syndrome. Adrenal uptake of the tracer in the patients with UCV-incidentalomas was not statistically different from that in the patients with Cushing's adenomas and had no relationship with hormonal values in either patient group. Tumor size on CT correlated with the levels of 24-hr urinary 17-OHCS (r = 0.75, p = 0.02) and plasma cortisol at 7:00 (r = 0.82, p = 0.007) in the patients with UCV-incidentalomas, but not in the patients with Cushing's adenomas. Although 3 UCV-incidentalomas increased slightly in size, none of 9 patients with UCV-incidentalomas has developed Cushing's syndrome for 4 to 52 months. These results suggest that the UCV-incidentaloma may be essentially different from the Cushing's adenoma and unlikely to develop Cushing's syndrome.

Topics: 17-Ketosteroids; Adenoma; Adrenal Cortex; Adrenal Cortex Neoplasms; Adrenocorticotropic Hormone; Adult; Aged; Aldosterone; Cushing Syndrome; Female; Humans; Hydrocortisone; Male; Middle Aged; Radionuclide Imaging; Regression Analysis

Here we report a case of a renin-producing adrenocortical carcinoma. A 57-year-old woman was referred to our hospital complaining of thirst and generalized muscle weakness. She was diagnosed as being hypertensive and diabetic with associated hypokalemia and she had a hard elastic mass with a diameter of 10 cm on the left side of her neck. An abdominal computed tomography scan revealed a suprarenal mass on the left side (8.5 x 8 x 6.5 cm). Endocrinological examination demonstrated a marked elevation in the patient's serum glucocorticoid and sex steroid hormones as well as plasma renin activity. Histological examination of a sample taken from the neck mass revealed a metastasis from an adrenal carcinoma, which was stained positively with antibodies against cytochrome P450 and renin, establishing the diagnosis of a renin-producing adrenocortical carcinoma. Trilostane was effective in reducing serum cortisol levels, but mitotane was ineffective.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; 3-Hydroxysteroid Dehydrogenases; Adrenal Cortex Neoplasms; Biopsy, Needle; Catecholamines; Cytochrome P-450 Enzyme System; Dihydrotestosterone; Enzyme Inhibitors; Female; Glucocorticoids; Gonadal Steroid Hormones; Humans; Hydrocortisone; Hypertension; Hypokalemia; Middle Aged; Mitotane; Muscle Weakness; Neoplasm Metastasis; Renin; Thirst; Tomography, X-Ray Computed

We report a case of preclinical Cushing's syndrome in a 54-year-old male associated with bilateral adrenocortical tumours. Physical findings and general laboratory data were unremarkable except for mild hypertension (158/90 mmHg) and impaired glucose tolerance. Endocrinological evaluation revealed the presence of autonomous cortisol secretion including unsuppressible serum cortisol by 8 mg dexamethasone test (11 microg/dl), high serum DHEA-S (3580 ng/ml, normal: 400-3500) and increased urinary 17-KS excretion (31.0-35.8 mg/day, normal: 5.8-21.3). CT scan demonstrated the presence of tumours in both adrenals and bilateral adrenalectomy was subsequently performed. Histological examination of the resected specimens revealed an adrenocortical carcinoma on the right side and an adenoma on the left side with noticeable cortical atrophy in non-neoplastic adrenals. Immunohistochemical study of steroidogenic enzymes demonstrated that all the steroidogenic enzymes involved in cortisol biosynthesis were expressed in both right and left adrenal tumours. Enzymatic activities of 21, 17alpha, 18, 11beta-hydroxylases were detected in both right and left adrenals except for the absence of 11beta-hydroxylase activity in the left adrenal adenoma. Results of in vitro tissue steroidogenesis examined in short-term tissue culture of the specimens revealed no significant differences between carcinoma and adenoma in cortisol production, but the production of adrenal androgens in carcinoma was significantly higher than that in adenoma, which may indicate the importance of evaluating adrenal androgen levels in patients with adrenocortical neoplasms.

Topics: 17-Ketosteroids; Adenoma; Adrenal Cortex Neoplasms; Adrenalectomy; Carcinoma; Culture Techniques; Cushing Syndrome; Dehydroepiandrosterone Sulfate; Dexamethasone; Glucocorticoids; Humans; Hydrocortisone; Immunohistochemistry; Male; Middle Aged; Neoplasms, Multiple Primary; Steroid Hydroxylases; Tomography, X-Ray Computed

Adrenocortical carcinoma (ACC) is a rare disease with a poor prognosis. It has been difficult to establish a strict treatment program for ACC, and better treatment alternatives and diagnostic tools must be sought. Even though surgery is the treatment of choice, the role of surgery in advanced disease has been questioned. Eighteen consecutive patients were treated at our unit over a 22-year period (1975-1997). All patients underwent surgery and were followed by our protocol, which includes urinary steroid profiles, clinical examinations, analysis of steroid hormones, and radiologic investigations. Twelve patients received mitotane with drug concentration measurements to deliver an effective, nontoxic dose. The median duration of mitotane treatment was 12 months. Few side effects were observed. Four patients with low-stage tumors underwent second-look operations with no pathologic findings. Five patients were subjected to repeat operations, and the mean duration of the disease-free interval before repeat surgery for these patients was 59 months. There was a significant positive correlation between the disease-free interval and the observed survival after repeat surgery. Eleven patients with intentionally curative surgery had their urinary steroid profiles tested several times postoperatively. For five patients preoperative urine samples were also available. Steroid profiles indicated recurrent disease despite normal radiologic findings in two of these five patients. The follow-up ranged from 6 weeks to 24 years. The predicted 5-year survival was 58% according to the Kaplan-Meier method. We conclude that monitoring serum concentrations of mitotane makes long-term treatment possible with few side effects; steroid profile analysis can be used for early detection of tumor recurrence; and repeat surgery for recurrence is of value for patients with long disease-free intervals.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Cortex Neoplasms; Adult; Aged; Antineoplastic Agents, Hormonal; Combined Modality Therapy; Disease-Free Survival; Female; Follow-Up Studies; Humans; Male; Middle Aged; Mitotane

Topics: 17-Ketosteroids; Adolescent; Adrenal Cortex Neoplasms; Age Factors; Child; Child, Preschool; Cushing Syndrome; Dehydroepiandrosterone; Environmental Pollutants; Female; Humans; Hypertension; Infant; Infant, Newborn; Male; Prognosis; Retrospective Studies; Sex Factors; Treatment Outcome; Virilism

Under analysis are results of an examination of 56 patients with hormonally non-active tumors of the adrenals, 48 of them were operated upon. In 20 patients (35%) they had clinical manifestations, 16 patients (28%) had an elevated level of the 17-HCS and 17-CS excretion in the 24-hours urine. Morphological investigations of the tumors in 20 patients have revealed clear cell adenomas of the cortical layer of the adrenal, in 19 patients--tumoral cysts, in 3 patients--true cysts, in 1 case-myelolipoma, in 1 case--ganglioneuromyelolipoma, in 4 cases--hormonally non-active carcinomas of the adrenal cortex. The authors propose to designate the new formations described as hormonally non-active tumors of the adrenals. In view of the absence of absolute methods for the identification of malignancy of the tumors the operative treatment of them is proposed.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adenoma; Adolescent; Adrenal Cortex Diseases; Adrenal Cortex Neoplasms; Adrenal Glands; Adult; Aged; Cysts; Humans; Intraoperative Complications; Middle Aged; Tomography, X-Ray Computed; Ultrasonography

A 28-year old female patient with virilization due to left adrenocortical adenoma was studied. The patient had clinical features of hyperandrogenism such as hirsutism and a low pitched voice, but not of hypercorticoidism. Plasma testosterone and dehydroepiandrosterone-sulfate (DHEA-S) were high. Although the basal plasma cortisol concentration and urinary excretion of 17-hydroxycorticosteroids (17-OHCS) were within the normal range, the absence of diurnal variation in plasma cortisol and loss of suppressibility by dexamethasone suggested constitutive secretion of cortisol by the tumor. Inappropriate cortisol secretion was also supported by blunted ACTH response to provocative stimuli. After successful removal of the left adrenal tumor, such endocrinological abnormalities were all normalized. Immunohistochemical analysis revealed that tumor cells were positively stained for C21 hydroxylase cytochrome P-450 (P-450C21) and P-450(11) beta which convert 17-hydroxy (OH) progesterone to cortisol as well as P-450SCC, 3 beta-hydroxysteroid dehydrogenase and P-450(17) alpha which are involved in testosterone biosynthesis. These findings suggest that adrenocortical adenoma secretes predominantly testosterone and constitutively cortisol in a young woman patient with virilization.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adenoma; Adrenal Cortex Neoplasms; Adult; Circadian Rhythm; Cortodoxone; Cushing Syndrome; Dehydroepiandrosterone; Dehydroepiandrosterone Sulfate; Dexamethasone; Female; Humans; Hydrocortisone; Immunohistochemistry; Japan; Testosterone

Adrenocortical carcinoma is a rare tumor with a poor prognosis. This work was aimed at analyzing the clinical outlook and treatment results of 52 patients with this disease.. This study included patients with adrenocortical carcinoma referred to the Department of Endocrinology at the Center of Postgraduate Medical Education (Warsaw, Poland) during the last 30 years. In 11 patients, the adrenal tumor was found incidentally by ultrasonographic scan. Hormonal examinations made it possible to define the endocrine activity of the tumors, whereas imaging techniques helped to determine their staging. Forty-eight patients underwent surgery, and 36 of them received mitotane. This drug was administered to 26 patients for a range of 10 months to 10 years; 13 patients received mitotane immediately after the operation, and 13 others after a delay. The patients with severe hypercorticism were pretreated before surgery with aminoglutethimide and mitotane.. The study comprised 10 men and 42 women; hormonally active tumors were diagnosed in 39 of them. Cushing's syndrome was the most frequent entity. At diagnosis, 17 cases were classified as localized disease, 15 as regional disease, and 20 as distant disease. Pretreatment with the inhibitors of steroidogenesis improved the survival perspectives in the early postoperative period. As of this writing, there were 12 survivors in the group of 26 patients treated by surgery and long term mitotane therapy and only 2 survivors of 7 patients treated with surgery only.. Surgery with immediate adjuvant long term mitotane administration was the most effective form of therapy for patients with adrenocortical carcinoma.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adolescent; Adrenal Cortex Neoplasms; Adrenocortical Carcinoma; Adrenocortical Hyperfunction; Adult; Aged; Aminoglutethimide; Chemotherapy, Adjuvant; Cushing Syndrome; Female; Follow-Up Studies; Growth Hormone; Growth Hormone-Releasing Hormone; Humans; Hydrocortisone; Male; Middle Aged; Mitotane; Neoplasm Staging; Survival Rate; Testosterone; Treatment Outcome

A 24-year-old man visited our hospital complaining of hypertension and headache. Endocrinological findings revealed no abnormalities except for a slight decrease in serum adrenocorticotropic hormone (ACTH), a slight increase in urine 17-ketosteroid (17-KS), and a marked increase in serum pregnenolone. Computed tomography and magnetic resonance imaging revealed a 3 x 3 cm mass in the right adrenal area and I131-aldosterol scintigraphy demonstrated a high absorption of the isotope in the right adrenal area. Vena cavography suggested a 2 x 2 cm tumor thrombus originating in the right adrenal. Under the diagnosis of the right adrenocortical carcinoma, adrenalectomy and removal of the tumor thrombus were performed. Both serum pregnenolone and urine 17-KS returned to the normal level within a week after the operation and blood pressure was well controlled without any medication 3 months after the operation. Thus, the tumor seemed to be endocrinologically active.

Topics: 17-Ketosteroids; Adrenal Cortex; Adrenal Cortex Neoplasms; Adrenalectomy; Adrenocorticotropic Hormone; Adult; Carcinoma; Humans; Iodine Radioisotopes; Magnetic Resonance Imaging; Male; Pregnenolone; Radionuclide Imaging; Tomography, X-Ray Computed; Venae Cavae

A 58-yr-old man presented with gynecomastia and elevated serum estrogens. The diagnosis of an estrogen-secreting adrenal tumor was made based upon the finding of a 4-cm left adrenal mass, elevated levels of estradiol in peripheral and left adrenal venous blood, and increased urinary 17-ketosteroids. In addition to marked elevations in estradiol and 17-ketosteroids there was an increased baseline level of 11-deoxycorticosterone and a slightly decreased level of 18-hydroxycorticosterone, suggesting the possibility of impaired P450c11 activity. The effect of ketoconazole administration (600 mg/day) for 4 weeks was studied. Urinary free cortisol and 17-ketosteroid excretion and serum testosterone levels fell acutely (1 week). Serum estradiol levels decreased gradually over the 4-week course. Plasma aldosterone levels were essentially unaltered and 18-hydroxcorticosterone levels fell gradually, but there were marked increases in 11-deoxycorticosterone and corticosterone. Coincident with the increase in 11-deoxycorticosterone there was an increase in blood pressure and a transient fall in serum potassium. We conclude that ketoconazole administration may result in a hypermineralocorticoid state. Therefore, the usefulness of ketoconazole therapy for steroid hormone-producing neoplasms will depend upon the individual tumor's steroidogenic profile.

Topics: 17-Ketosteroids; Adrenal Cortex Neoplasms; Aldosterone; Androgen-Insensitivity Syndrome; Corticosterone; Desoxycorticosterone; Estradiol; Estrogens; Humans; Hydrocortisone; Ketoconazole; Male; Middle Aged; Testosterone

We present nine cases of primary adrenocortical carcinoma, collected in our department of medicine and endocrinology over 25 years. In our patients, the most dependable tumor marker was urinary excretion of tetra-hydro-ll-deoxycortisol (THS), and elevated values were found in all cases where it was determined. In addition to surgical treatment, medication with o,p'-DDD was found to be of value for some of the patients. Median tumor weight was 487 g (118-2,085 g). Prognosis is difficult to predict. Median survival time after diagnosis was 34 months, but varied from three to 266 months.

Topics: 17-Ketosteroids; Adrenal Cortex Neoplasms; Adult; Aged; Biomarkers, Tumor; Cortodoxone; Cushing Syndrome; Female; Humans; Hydrocortisone; Male; Middle Aged; Mitotane; Neoplasm Metastasis; Prognosis

Topics: 17-Ketosteroids; Adenoma; Adrenal Cortex Neoplasms; Diagnosis, Differential; Female; Humans; Middle Aged

Ten patients with adrenal cortical carcinoma were treated from 1966 to 1986. There were 7 males and 3 females. The typical clinical manifestations, marked increase of 17-ketosteroid, 17-hydroxycorticoids and DHA, and negative dexamethasone suppression test were essential for the diagnosis. Of the ten patients, eight had secretive function and their 17-ketosteroid and 17-hydroxycorticoids varied from 36.8-93 mg% and 32.5-150 mg%, respectively. DHA was measured in 5 cases with the result of 6.95-44mg%. Those without secretive functions or obvious endocrine disturbances were usually misdiagnosed as kidney tumor, splenomagaly, liver tumor or pancreatic mass. Wood had summarized that nonsecretive ACC patients commonly had fever, pain, exhaustion syndrome (emaciation, fatigue, perspiration, anorexia), mass and distant metastasis. Adrenal scan, IVU, abdominal aortic arteriography, retroperitoneal pneumography and CT were helpful in localization. The differential diagnosis between ACC and adenoma by pathology was difficult. It is generally agreed that if the mass is larger than 100 grams, capsulated, having blood or lymphatic vessel invasion, hemorrhage, necrosis and calcification or even distant metastasis, malignant tumor should be considered. Surgical removal of the tumor is the only effective treatment. For advanced or recurrent lesions, selective adrenal artery thrombosis could be used. One of the ten patients was thus treated by this facilitated subsequent surgery. Postoperative chemotherapy, such as O.P-DDD, might be used in some cases.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Cortex Neoplasms; Adult; Aged; Carcinoma; Dehydroepiandrosterone; Diagnosis, Differential; Female; Humans; Male; Middle Aged

Adrenocortical carcinoma (ACC) is a rare disorder with an estimated incidence of only 0.023 percent of all malignancies. In most cases, Cushing's syndrome and virilization or feminization due to abnormal steroid production by the tumor rapidly lead to the diagnosis. Occasionally, the tumor produces an excessive amount of mineralocorticoids only and ACC can be revealed by an isolated syndrome of primary aldosteronism. Out of 100 cases of tumoral primary aldosteronism studied from 1977 to 1987, we observed 4 ACC and 96 Conn's adenomas (CONN). When primary aldosteronism was diagnosed, ACC and CONN had same clinical features, although hypokalemia in ACC was more profound: 2.2 +/- 0.76 mmol/l (1.4 to 3.2) compared to 2.9 +/- 0.5 (1.6 to 4.2) in CONN. Mean supine plasma aldosterone levels, plasma renin and aldosterone responses to the upright posture or to serum saline infusion, cortisol at 8 a.m. were not different in patients with ACC from those observed in patients with CONN. 24 hours urinary cortisol excretion and 17-ketosteroids excretion were highly increased in three out four patients with ACC. Clinical, biological and hormonal investigations were therefore not sufficient to diagnose malignant tumoral primary aldosteronism. Systematic computed tomographic scanning allowed to differentiate carcinomas from adenomas on the following criteria: ACC showed enlarged tumor size that was always above 30 mm in diameter, whereas the largest CONN measured 20 mm.ACC appeared as an heterogeneous tumor with the presence of internal calcifications in each case of ACC, that were diagnosed both on ultrasound and CT scan, whereas none of the CONN showed any calcification, using the same screening procedure.(ABSTRACT TRUNCATED AT 250 WORDS)

Topics: 17-Ketosteroids; Adolescent; Adrenal Cortex Neoplasms; Adult; Aldosterone; Calcinosis; Female; Humans; Hydrocortisone; Hyperaldosteronism; Male; Middle Aged; Posture; Potassium; Renin

A 36-year-old woman who had experienced two pregnancies consulted our hospital, because of scant menses and virilization. A 24-hour excretion of 17-ketosteroids and 17-hydroxy-corticosteroids demonstrated a decrease in 11-hydroxylase. A computed tomogram showed a huge inhomogenous tumefaction in the left adrenal. Left selective renal angiography revealed a large adrenal tumefaction. Selective adrenal venous samplings revealed that testosterone and dehydroepiandrosterone (DHA) were produced in response to stimulation by 0.25 mg exogenous adrenocorticotropic hormone (ACTH). After left adrenalectomy was performed, a diagnosis of adrenocortical carcinoma was made by pathological examination. This is the first report of a patient with a virilizing adrenocortical carcinoma, which produced testosterone and DHA in response to exogenous ACTH stimulation.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Cortex Neoplasms; Adrenocorticotropic Hormone; Adult; Carcinoma; Dehydroepiandrosterone; Female; Humans; Testosterone; Tomography, X-Ray Computed; Virilism

The analysis of 24-h excretion profiles of urinary steroids in 18 patients suffering from Cushing's syndrome or adrenocortical tumors revealed typical patterns when compared to 37 healthy control persons, 24 patients with obesity, and 6 patients with hirsutism. The validation of eight criteria--increased excretion of free cortisol, 6 beta-hydroxycortisol, 20 alpha-dihydrocortisol, 11 beta-hydroxyandrosterone, and 3 beta-hydroxy-5-en steroids, decreased ratio of tetrahydrocortisone (THE) to tetrahydrocortisol (THF), and increased ratios of THF to allotetrahydrocortisol (a-THF) and metabolites of androgens (AM) to metabolites of cortisol (CM)--afforded reliable detection of disorders in steroid biosynthesis. The analysis of urinary steroid profiles can therefore be recommended as a screening procedure in patients with clinical symptoms of disorders in steroid production and/or metabolism.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adolescent; Adrenal Cortex Neoplasms; Adrenocorticotropic Hormone; Adult; Child; Cushing Syndrome; Female; Humans; Hydrocortisone; Male

A 58-year-old white woman with hypertension and severe hypokalemia was found to have a carcinoma of the left adrenal gland. Plasma renin activity was constantly under the normal limit, while plasma aldosterone levels were pathologically elevated. Plasma cortisol (8:00 a.m.) and excretion rates of urinary free cortisol were within the normal range. After an adrenalectomy, relapsing excessive aldosterone secretion was successfully treated with opDDD (Lysodrene). Ten months after the diagnosis was established, the patient died from a bleeding liver metastasis.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Cortex Neoplasms; Aldosterone; Combined Modality Therapy; Female; Humans; Hydrocortisone; Hyperaldosteronism; Middle Aged; Paraneoplastic Endocrine Syndromes; Postoperative Complications; Renin

The experience with adrenocortical neoplasms in childhood is reviewed. During three decades, ten children with adrenocortical neoplasms were seen at the authors' institution. The literature was reviewed, and 209 patients 16.5 years or younger were found. All ten patients at Vanderbilt University Hospital (VUH) presented with endocrine manifestations of the tumor. Three patients had Cushing's, two patients had virilization, and five patients had features of both. In the literature, virilization, alone or with Cushing's, was the most common mode of presentation. Feminizing tumors were uncommon and nonfunctional tumors rare. The majority of patients were female. Features associated with malignancy included 17-KS levels greater than 40 mg/24 hr, diameter greater than 6 cm, weight greater than 500 g, and histologic evidence of diffuse growth pattern, vascular invasion, and tumor cell necrosis. Although uncommon, adrenocortical neoplasms should be suspected in children with Cushing's, virilization, feminization, or a combination of these. There has been an increased incidence reported in patients with hemihypertrophy, Beckwith-Wiedemann syndrome, hemangiomas, and nevi. Following endocrinologic evaluation, imaging studies should be performed. CT scan appears to be the most useful diagnostic tool. A posterior operative approach is recommended for suspected adenomas. An anterior thoracoabdominal approach is favored for suspected malignancies with uncompromised en bloc resection. There is no evidence that adjuvant therapy provides any additional benefit.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adenoma; Adolescent; Adrenal Cortex Neoplasms; Carcinoma; Child; Child, Preschool; Cushing Syndrome; Female; Hirsutism; Humans; Hypertrophy; Infant; Male; Prognosis

Nine cases of adrenocortical tumor are presented, six were males. Four were less than three years and five were between 5 and 10 years of age. Clinical virilization was found in 8 children, one had only signs of hypercortisolism and another showed signs of virilization and hypercortisolism simultaneously. Urinary 17-KS and 17-OHCS were high in all patients. Plasma levels of testosterone and of the other adrenal androgens were high in all the cases tested. Plasma level of cortisol was elevated only in few cases. In two out of five cases steroids were only partially suppressed by dexamethasone. Computed tomography and abdominal sonography have been useful tools for the localization of the tumour. The resection of the tumour, independent of histopathological diagnosis, led to a complete normalization of the clinical and hormonal picture in eight cases evaluated at a distance of 2 months 10 years after surgery. In one case a hepatic metastasis was observed and removed three years after surgery.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Cortex Neoplasms; Adrenocortical Hyperfunction; Child; Child, Preschool; Cortisone; Humans; Infant; Male; Virilism

A 47-year-old woman affected by Cushing's syndrome due to an adrenal adenoma is described. An altered but rhythmometrically apparent cortisol secretory rhythm was detected using the single-cosinor computation. In fact serum cortisol levels and urinary excretion of 17-OHCS were elevated in the PM hours, particularly between 14:00-18.00 h and 18:00-22:00 h, and normal between 02:00-10:00 h. The patient was cured by unilateral adrenalectomy and one year later the circadian rhythm of corticosteroids secretion was investigated again. A normal rhythm of cortisol secretion and of 17-OHCS urinary excretion was found. Though it may be hypothesized that factors intrinsic to the tumoral adrenal cells were responsible for the rhythmic, but phase-shifted, hormonal release, the cause of the persistent and abnormal cortisol secretory rhythm is unknown.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adenoma; Adrenal Cortex Neoplasms; Adrenalectomy; Adrenocorticotropic Hormone; Circadian Rhythm; Cushing Syndrome; Female; Glucocorticoids; Humans; Hydrocortisone; Hypothalamo-Hypophyseal System; Middle Aged; Pituitary-Adrenal System; Time Factors

A case of adrenal carcinoma with Cushing's syndrome was presented. Endocrinological and morphological investigations disclosed the presence of a functional adrenal carcinoma. This case was characterized by its unusual urinary 17-ketosteroid (17-KS) fractionation, i.e. a marked elevation of 17-KS was accompanied by the increments of etiocholanolone, but not of dehydroepiandrosterone (DHEA) or androsterone. Measurements of the plasma adrenocorticosteroids revealed normal DHEA and DHEA-S (sulfate) levels, moderately increased 17-OH-pregnenolone, and markedly increased (less than 100 times the normal) 11-deoxycortisol (cpd S). Therefore, it seems plausible that the normal urinary DHEA level in this patient would have occurred as a result of remarkably low C17-20 lyase activity sufficient to hamper DHEA production, and that markedly increased etiocholanolone might possibly have been converted from cpd S as well as from DHEA and androstenedione through 5 beta-reduction.

Topics: 17-alpha-Hydroxypregnenolone; 17-Ketosteroids; Adrenal Cortex Neoplasms; Adult; Androstenedione; Androsterone; Cortodoxone; Cushing Syndrome; Dehydroepiandrosterone; Dehydroepiandrosterone Sulfate; Etiocholanolone; Humans; Male; Tomography, X-Ray Computed

Seventeen patients with adrenal adenoma causing Cushing's syndrome, eight patients with Cushing's disease due to hypersecretion of ACTH, and five patients with primary aldosteronism due to an aldosteronoma were studied for their computed tomographic (CT) patterns, hormonal profiles, and macroscopic and microscopic findings of the adrenal gland. Black (or brown) adrenal adenomas were found in 71% of the patients with Cushing's syndrome, but not in patients with aldosteronoma. The adrenal tissue of patients with Cushing's disease was predominantly yellow. The number of compact cells was larger in black or brown adenomas than in yellow tumors or hyperplastic adrenal tissue. In patients with Cushing's syndrome, urinary excretion of 17-ketosteroids (17-KS) and serum aldosterone concentrations were lower in those with black or brown adenomas than in those with yellow adenomas (P less than 0.05). Patients with Cushing's disease had even higher 17-KS and serum aldosterone levels. No difference was found in serum cortisol concentrations and dexamethasone suppressibility in two types of adenomas causing Cushing's syndrome. Visual estimation of radiological density of the adrenal tissue relative to the kidney on CT scan and quantitative measurement of it by CT number revealed a difference between the two types of adrenal tumors causing Cushing's syndrome. Adrenal tumors with decreased density on CT scan were yellow adenomas with predominantly clear cells, and those with equal or increased density were black or brown adenomas with predominantly compact cells. All aldosteronomas had decreased density and consisted of clear cells. It is suggested that black or brown adenomas of the adrenal gland have higher radiological density and accompanying lower serum aldosterone and urinary 17-KS levels than ordinary yellow tumors. The abundance of compact cells may have some significance for the development of this particular type of adrenal tumor.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adenoma; Adolescent; Adrenal Cortex Neoplasms; Adult; Aldosterone; Color; Cushing Syndrome; Female; Humans; Hydrocortisone; Male; Microscopy, Electron; Middle Aged; Tomography, X-Ray Computed

With the wider application of increasingly sensitive computed tomographic scans, more adrenal masses will be discovered incidentally. Because benign lesions of the adrenal are much commoner than malignant ones, an approach is needed to determine which incidentally discovered masses should be removed. The history and physical examination may guide the evaluation. Imaging studies and needle biopsies have limited value. If the history and physical findings do not suggest a diagnosis, an approach using the size of the mass, results of any cyst puncture, and a biochemical assessment may determine which patients should have surgery. This approach is based on the relative prevalence of benign and malignant clinically silent adrenal tumors.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adenoma; Adrenal Cortex Neoplasms; Adrenal Gland Neoplasms; Biopsy, Needle; Carcinoma; Diagnosis, Differential; Female; Humans; Male; Pheochromocytoma; Sex Factors; Tomography, X-Ray Computed

Topics: 17-Ketosteroids; Adenocarcinoma; Adrenal Cortex Neoplasms; Adult; Carcinoma; Cushing Syndrome; Female; Humans; Middle Aged

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adenoma; Adrenal Cortex Neoplasms; Adult; Aged; Female; Humans; Hydrocortisone; Male

Topics: 17-Ketosteroids; Adrenal Cortex Neoplasms; Adrenal Glands; Adrenal Hyperplasia, Congenital; Atrophy; Carcinoma; Child; Female; Humans; Virilism

We report the hormonal and radiological evaluation of two cases of adrenal cortical adenomas that secreted testosterone exclusively. We discuss some of the pitfalls in the diagnosis of this lesion, and summarize the current knowledge of the characteristic hormonal features in the two cases and the 12 cases previously reported.

Topics: 17-Ketosteroids; Adenoma; Adrenal Cortex Neoplasms; Adult; Dexamethasone; Estradiol; Female; Follicle Stimulating Hormone; Humans; Hydrocortisone; Luteinizing Hormone; Male; Middle Aged; Pregnancy; Testosterone

Twenty-one patients with adrenocortical carcinoma (ACC) diagnosed at three hospitals over a ten-year period were reviewed for clinical and pathologic features that might have therapeutic implications. Depending upon the extent of cellular pleomorphism, ACC could be defined as anaplastic or differentiated. Anaplastic ACC occurred more often in male patients, produced more frequent cutaneous metastases (P = 0.01), and was associated with a lack of clinical or laboratory evidence of hormone production (P = 0.01). In contrast, differentiated ACC usually occurred in women and produced clinical or laboratory evidence of hormonal excess. Median survival time of patients with anaplastic ACC was only five months, while median survival time of those with differentiated ACC was 40 months (P = 0.005). Patients with differentiated ACC survived for long periods, even with metastatic disease. Three of 5 such patients had objective responses to o,p'-DDD (Mitotane) therapy. The histopathology of ACC is an important prognostic factor and should be considered in the design of future therapeutic trials.

Topics: 17-Ketosteroids; Adolescent; Adrenal Cortex Neoplasms; Adult; Aged; Carcinoma; Cell Transformation, Neoplastic; Female; Humans; Hydrocortisone; Hydroxysteroids; Male; Middle Aged; Prognosis; Retrospective Studies; Time Factors

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Cortex Neoplasms; Adrenalectomy; Adult; Female; Humans; Middle Aged; Mitotane

A 37-year-old male with adrenocortical tumor presenting marked gynecomastia and impotence as initial symptoms was experienced. Abnormal elevations in urinary 17-KS, 17-OHCS and blood estrogen levels were noted. Total surgical removal of the tumor weighing 800g was performed. Postoperatively the gynecomastia disappeared and he regained libido with no signs of recurrence one year later.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Cortex; Adrenal Cortex Neoplasms; Adult; Estrogens; Feminization; Gynecomastia; Humans; Male

Four post-menopausal women had Cushing's syndrome due to adrenal cortical carcinomas. Comprehensive analyses of blood and urinary steroids showed that although the steroid profiles differed between patients, the pattern in each patient remained almost constant as the disease progressed, or remitted due to therapy. Elevations of serum testosterone and oestradiol were commensurate with the extent of virilisation, and the urinary output of aldosterone was associated with the severity of hypertension. A new finding was that all had substantially increased urinary free deoxycorticosterone. Complete surgical removal of the primary tumours was impossible but when most of the tumour tissue was removed, full clinical and biochemical remissions were obtained for a short time in 2 patients. One patient obtained a clinical and biochemical remission from op'DDD. In another patient the drug caused reduction both in blood pressure and in urinary aldosterone excretion, but there were unpleasant side effects. A third patient could not tolerate op'DDD. Metyrapone therapy produced neither clinical nor biochemical improvement in 3 patients. The mean duration of survival was 17 months after the first symptoms and 10 months from the date of operation. Despite advances in drug therapy, adrenal cortical carcinoma remains a lethal disease. Biochemical screening of multiple steroids offers a means of early diagnosis and disease monitoring. Extensive surgical removal of the tumour offers the best chance of a clinical and biochemical remission.

Topics: 17-Ketosteroids; Adrenal Cortex Neoplasms; Adrenocorticotropic Hormone; Aldosterone; Carcinoma; Cortodoxone; Cushing Syndrome; Desoxycorticosterone; Electrolytes; Estradiol; Female; Humans; Hydrocortisone; Luteinizing Hormone; Metyrapone; Middle Aged; Mitotane; Pregnanetriol; Testosterone

A 31 years old man with an adrenocortical carcinoma was studied. Clinically he had a bilateral and recidiving gynecomastia and showed high urinary oestrogens, 17 cetosteroids, tetra-hydro-desoxy-cortisol and pregnandiol excretion with normal cortisol production. A partial increase on ACTH, no suppression on dexamethasone and no variation on HCG administration were observed. The surgical resection of the tumor normalized this urinary excretion. The serum dehydro-epiandrosterone (DHEA) and sulfate (DHEAS), oestrone, oestradiol, androstenedione (A) levels were greatly elevated. No variations of the cortisol, A, DHEA and DHEAS was noted after ACTH injection. In vitro the lack of ACTH's action was related to an anomaly of ACTH receptor with normal protein kinase activity.

Topics: 17-Ketosteroids; Adrenal Cortex Neoplasms; Adrenocorticotropic Hormone; Adult; Androstenedione; Carcinoma; Chorionic Gonadotropin; Dehydroepiandrosterone; Dexamethasone; Estrogens; Feminization; Humans; Hydrocortisone; Male; Pregnanediol; Tetrahydrocortisol

A 3-year-old girl affected by a virilizing tumor of the adrenal gland, without significant elevation in the levels of 17 ketosteroids (17-KS) urinary excretion, was studied clinically. Her symptoms started abruptyly at the age of 2, with progressive enlargement of the clitoris and the appearance of pubic hair. In various tests, the 17-KS levels barely exceeded the upper normal limits and at times remained within normal limits. The retropneumoperitoneum X-ray suggested an enlargement of the right adrenal gland and the presence of a neoplasm, which was actually discovered during surgery. Histopathological examination revealed a well-defined neoplasm, without capsule invasion and with accentuated cell polymorphism. Histoenzymology showed that the tissue lacked the enzymatic system involving 3 beta-hydroxysteroid dehydrogenase (3 beta-HSD). Indoxylesterase (I.EST-A) activity identified the tumor as originating from the internal layers of the adrenal cortex. The histochemical findings were correlated to the clinical picture and the levels of urinary 17-KS.

Topics: 17-Ketosteroids; 3-Hydroxysteroid Dehydrogenases; Adrenal Cortex Neoplasms; Child, Preschool; Clitoris; Female; Hair; Histocytochemistry; Humans; Hypertrophy; Isomerases; Virilism; Vulva

A review of adrenal cortical carcinoma in childhood is presented, including endocrinologic aspects. Electron microscopic features are presented, as well as the finding of hypertrophied spermatic tubules. The importance of serial steroidal determinations is stressed as a "tumor marker" that may help in early detection of recurrent disease and possibly increased long-term survival.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Cortex Neoplasms; Child, Preschool; Female; Gonadal Steroid Hormones; Humans; Hypertrophy; Male; Seminiferous Tubules; Testis; Virilism

Topics: 17-Ketosteroids; Adrenal Cortex Neoplasms; Adult; Carcinoma; Female; Humans; Lung Neoplasms; Neoplasm Metastasis; Pleural Effusion

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adolescent; Adrenal Cortex Neoplasms; Adult; Aged; Child; Child, Preschool; Female; Humans; Infant; Male; Middle Aged

We report on four patients, aged seven months to seven years, with virilizing adrenal cortical tumors. Diagnosis was made by clinical and laboratory data. Tumor localisation was achieved preoperatively in three patients. In two patients both androgen and glucocorticoid excretion were elevated. In three patients histological examination showed a carcinoma, in one patient an adenoma was suggested. There were no metastases. In two patients surgical removal was followed by cytostatic therapy, in one of them additional irradiation treatment was carried out. This child died 10 1/2 years later from a metastasizing renal cell carcinoma which originated from the kidney being situated in the irradiated area.

Topics: 17-Ketosteroids; Adenoma; Adrenal Cortex Neoplasms; Adrenal Gland Neoplasms; Child; Female; Glucocorticoids; Humans; Infant; Male

Two cases of androgen secreting adrenocortical carcinoma have been described by light and electron microscopy. The histological and ultrastructural features of the tumour cells were similar to those of compact cells of zona reticularis and to those described in virilizing adenomas. They possess numerous mitochondria with lamellar and tubular cristae, abundant smooth endoplasmic reticulum, lipofuscin bodies and scanty lipid. Irregularly shaped, crenated mitochondria, with outpouchings of the outer limiting membrane have also been observed. The clusters of neoplastic cells were surrounded by basement membrane which demonstrated a focal discontinuity, probably reflecting malignancy of the tumours. Hyperplasia of smooth endoplasmic reticulum and the presence of outpouchings of the mitochondrial outer limiting membrane might be the morphological manifestation of endocrine activity of the tumours.

Topics: 17-Ketosteroids; Adenoma; Adolescent; Adrenal Cortex Neoplasms; Adrenal Gland Neoplasms; Adrenalectomy; Androgens; Child, Preschool; Female; Humans; Liver Neoplasms; Male; Neoplasm Metastasis

A review of the literature reveals 230 cases of adrenal cortical tumors in children. We add an additional 7 cases. Signs, symptoms, laboratory studies, and treatment of this rare rate tumor are discussed.

Topics: 17-Ketosteroids; Adrenal Cortex Neoplasms; Age Determination by Skeleton; Angiography; Child, Preschool; Female; Humans; Infant; Male; Ultrasonography; Urography

Seven patients with Cushing's syndrome were treated with trilostane (WIN 24,540) 4 alpha,5-epoxy-17 beta-hydroxy-3-oxo-5 alpha-androstane-2 alpha-carbonitrile), an inhibitor of adrenal steroid biosynthesis. Trilostane treatment reduced steroid biosynthesis and it also improved biochemical manifestations of the disease in all of the patients treated. The average cortisol secretory rate decreased significantly with treatment, from 47.1 to 23.4 mg/24 h (P less than 0.005), and urinary 17-hydroxycorticosteroids decreased from 15.7 to 8.7 mg/24 h (P less than 0.01). Urinary free cortisol excretion decreased from 277 to 88 microgram/24 h (P less than 0.01), and 0800 h plasma cortisol levels declined from 25.0 to 12.0 microgram/dl (P less than 0.05). Conversely, dehydroepiandrosterone sulfate excretion in urine increased from 1.3 to 5.8 mg/24 h (P less than 0.0025) and in plasma increased from 162 mg/24 h (P less than 0.025). Plasma and urinary free dehydroepiandrosterone increased 2-fold. Urinary 17-ketosteroid excretion increased from 18 to 43 mg/24 h (P less than 0.001). A significant reduction in urinary excretion of tetrahydroaldosterone, tetrahydrodeoxycorticosterone, and 18-hydroxytetrahydrodeoxycorticosterone was observed with treatment. Inhibition of steroid biosynthesis was accompanied by a 2-fold increase in PRA and no change in serum cholesterol levels. Mean arterial blood pressure decreased with treatment from 109 to 97 mm Hg (P less than 0.005), and fasting blood sugar decreased from 117 to 98 mg/dl (P less than 0.005), accompanied by rise in plasma potassium levels from 3.8 to 4.3 milliequivalents/liter (P less than 0.025). Two patients on long term therapy also showed an improvement in clinical features of their disease. There were no significant treatment-related carcinoma, simultaneously producing both an excessive amount of cortisol and ACTH, is described. It is concluded that trilostane is an effective inhibitor of 3 beta-hydroxysteroid dehydrogenase enzyme system in human adrenal gland; it inhibits biosynthesis of cortisol and it is useful in the treatment of Cushing's syndrome.

Topics: 17-Ketosteroids; Adrenal Cortex Hormones; Adrenal Cortex Neoplasms; Adrenocorticotropic Hormone; Adult; Blood Pressure; Carcinoma; Cushing Syndrome; Dehydroepiandrosterone; Dehydroepiandrosterone Sulfate; Dihydrotestosterone; Female; Humans; Hydrocortisone; Kinetics; Male; Middle Aged; Mineralocorticoids; Potassium; Renin

A patient with adrenocortical carcinoma had three major endocrine abnormalities attributable directly to the tumor: hypercortisolism (Cushing's syndrome), hyperestrogenism (feminization), and hypercalcemia (pseudohyperparathyroidism). There were higher levels of immunoreactive parathyroid hormone in venous effluent from the tumor or its abdominal metastases compared to that found in the veins draining the parathyroid glands. This, together with the presence of normal parathyroid glands on autopsy, established the diagnosis of pseudohyperparathyroidism as the cause of hypercalcemia in this patient.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Cortex Neoplasms; Adrenal Gland Neoplasms; Adult; Aminoglutethimide; Androstenedione; Cushing Syndrome; Dexamethasone; Estrogens; Feminization; Humans; Hyperparathyroidism; Male; Metyrapone; Mitotane; Paraneoplastic Endocrine Syndromes; Parathyroid Hormone; Testosterone

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Cortex Neoplasms; Adrenal Gland Neoplasms; Adrenal Hyperplasia, Congenital; Child; Chromatography, Gas; Cushing Syndrome; Female; Humans; Male

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.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adenoma; Adolescent; Adrenal Cortex Neoplasms; Adrenal Gland Neoplasms; Adrenocortical Hyperfunction; Angiography; Child; Child, Preschool; Cushing Syndrome; Dehydroepiandrosterone; Female; Feminization; Humans; Male; Mitotane; Urography; Virilism

A virilizing adrenocortical adenoma was demonstrated in a yound female. Urinary 17-ketosteroid excretion and subfractions, plasma testosterone level and urinary 17-ketogenic steroid excretion were markedly increased. Dehydroepiandrosterone was the main constituent of the androgen excess. Otherwise the adrenocortical function was found to be normal as evaluated from measurements of cortisol, corticosterone and their metabolites under basal conditions and during dynamic tests. The androgen excess showed an unexpected response to trophic hormones. Human chorionic gonadotrophin stimulation resulted in a pronounced increase in androgen production, whereas no gonadotrophin-dependency could be demonstrated by means of the oestrogen suppression test. Similarly, no corticotrophin-dependency could be demonstrated by corticotrophin stimulation and suppression tests. Removal of the tumour resulted in normalization of the androgen production and no abnormal response upon human chorionic gonadotrophin administration could now be found. The inappropriate response of tumours to trophic hormones is discussed. It is concluded that the reliability of stimulation and suppression tests in determining the site of excessive androgen production should be accepted with reservation.

Topics: 11-Hydroxycorticosteroids; 17-Ketosteroids; Adenoma; Adrenal Cortex Neoplasms; Adrenal Gland Neoplasms; Adrenocorticotropic Hormone; Adult; Androgens; Androsterone; Chorionic Gonadotropin; Corticosterone; Dehydroepiandrosterone; Dexamethasone; Etiocholanolone; Female; Humans; Hydrocortisone; Hydroxycorticosteroids; Ovary; Pregnancy; Stimulation, Chemical; Testosterone

Topics: 17-Ketosteroids; Adolescent; Adrenal Cortex Neoplasms; Adult; Amenorrhea; Androgens; Androstenedione; Carcinoma; Chorionic Gonadotropin; Clomiphene; Dexamethasone; Estrogens; Ethinyl Estradiol; Female; Hirsutism; Humans; Hydrocortisone; Ovarian Cysts; Ovarian Neoplasms; Pregnancy; Sertoli-Leydig Cell Tumor; Testosterone; Virilism

Topics: 17-Ketosteroids; Adolescent; Adrenal Cortex Neoplasms; Adult; Age Factors; Aged; Androsterone; Chemical Phenomena; Chemistry; Child; Child, Preschool; Chromatography, Gas; Dehydroepiandrosterone; Etiocholanolone; Female; Humans; Infant; Infant, Newborn; Male; Middle Aged; Pregnanediol; Pregnanes; Pregnanetriol; Puberty; Sex Factors; Steroids; Testosterone

Topics: 17-Ketosteroids; Adrenal Cortex Neoplasms; Adrenal Gland Neoplasms; Female; Hormones, Ectopic; Humans; Hydrocortisone; Middle Aged; Paraneoplastic Endocrine Syndromes; Testosterone; Virilism

Topics: 17-Ketosteroids; 46, XX Disorders of Sex Development; Adenoma; Adrenal Cortex; Adrenal Cortex Hormones; Adrenal Cortex Neoplasms; Adrenocortical Adenoma; Adrenogenital Syndrome

Topics: 17-Ketosteroids; Adrenal Cortex Hormones; Adrenal Cortex Neoplasms; Adrenocorticotropic Hormone; Aged; Cortisone; Estrogens; Feminization; Gonadotropins; Gynecomastia; Humans; Male

Topics: 17-Ketosteroids; Adenoma; Adrenal Cortex; Adrenal Cortex Neoplasms; Adrenal Gland Neoplasms; Child; Cortisone; Desoxycorticosterone; Hydrocortisone; Neoplasms; Surgical Procedures, Operative

Topics: 17-Ketosteroids; Adrenal Cortex; Adrenal Cortex Diseases; Adrenal Cortex Neoplasms; Child; Humans; Neoplasms; Puberty; Puberty, Precocious; Sexual Maturation

Topics: 17-Ketosteroids; Adrenal Cortex; Adrenal Cortex Neoplasms; Adrenocortical Carcinoma; Humans; Ketosteroids; Male; Propiophenones

Topics: 17-Ketosteroids; Adrenal Cortex; Adrenal Cortex Neoplasms; Body Fluids; Cushing Syndrome; Humans; Urinary Tract

Topics: 17-Ketosteroids; Adenoma; Adrenal Cortex; Adrenal Cortex Neoplasms; Adrenocortical Adenoma; Estrogens; Female; Humans; Virilism

Topics: 17-Ketosteroids; Adrenal Cortex; Adrenal Cortex Neoplasms; Adrenocortical Carcinoma; Dose Fractionation, Radiation; Female; Humans; Steroids; Urine; Vaginal Neoplasms

Topics: 17-Ketosteroids; Adrenal Cortex; Adrenal Cortex Hormones; Adrenal Cortex Neoplasms; Adrenocorticotropic Hormone; Neoplasm Recurrence, Local; Neoplasms; Steroids

Topics: 17-Ketosteroids; Adrenal Cortex; Adrenal Cortex Neoplasms; Adrenal Gland Neoplasms; Body Fluids; Cortisone; Steroids

Topics: 17-Ketosteroids; Adrenal Cortex; Adrenal Cortex Hormones; Adrenal Cortex Neoplasms; Hyperplasia; Steroids

Topics: 17-Ketosteroids; Adrenal Cortex Neoplasms; Adrenal Gland Neoplasms; Adrenal Glands; Disorders of Sex Development; Humans; Neoplasms