[Diagnosis and management of adrenal cortex neoplasms].
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Although uncommon, testosterone producing neoplasms of the ovary and the adrenal cortex occur periodically. Forty-seven instances involving the adrenal gland have been published since 1925; five, including the present patient, have occurred since 1981. Confusion concerning the site of the androgen secretion has been generated in the past because of limited roentgenographic study and the poor predicability of selective suppression or stimulating testing of the adrenal gland and the ovary. Of the 22 patients with testosterone producing neoplasms of the adrenal cortex reported since 1973, 45 per cent underwent needless ovarian surgical treatment prior to the correct diagnosis. By recognizing the significant correlation between a serum testosterone of 200 nanograms per deciliter or greater, a serum dehydroepiandrostenone of greater than 6,600 nanograms per milliliter coupled with a thorough pelvic examination, pelvic ultrasound and a more liberal use of high resolution computerized tomography of the adrenal gland, the correct diagnosis can be made. The patient presented herein illustrates the pitfalls inherent in the management of a patient with this problem.
The key to correct diagnosis of abdominal masses in children is awareness of all the possibilities. Three children with abdominal masses of uncommon urologic origin are described. The lesions were adrenal cortical carcinoma, a pancake pelvic kidney and a non-functioning hydronephrotic segment of a horseshoe kidney. Management of these patients is discussed and the literature is reviewed.
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The authors identified 132 patients who died with a documented diagnosis of familial adenomatous polyposis (FAP). A review of the medical records, autopsy reports, and in-depth discussion with local physicians and well-informed family members was performed. It was impossible, even after the review, to ascertain the exact cause of death in 22 patients. In the remaining patients, the cause of death was as follows: metastatic colorectal carcinoma, 64 patients (58.2 percent), (colon, 49 [44.5 percent], rectal, 15 [13.6 percent]); desmoid tumors, 12 (10.9 percent); periampullary carcinoma, 9 (8.2 percent); brain tumors, 8 (7.3 percent); perioperative mortalities, 5 (4.5 percent); adrenal carcinoma, 1 (0.9 percent); and abdominal carcinomatosis, 1 (0.9 percent). Ten patients died of causes not related to FAP. The major causes of death in 36 patients who underwent prophylactic colectomy were desmoid tumor and periampullary malignancy. This finding underscores the importance of lifelong surveillance and periodic endoscopic evaluation in patients with FAP.
Two patients presented to hospital with clinical features of acute retroperitoneal haemorrhage. In each case, laparotomy revealed massive adrenal haemorrhage, and histological evidence of underlying neoplasia was eventually found.
The study describes the effects of ACTH, prolactin and other protein hormones on the synthesis and secretion of steroid hormones by tissue from a feminising adrenocortical carcinoma removed from a post-menopausal female. Steroid production by the tissue was determined by high resolution-mass fragmentography and by radioimmunoassay. Prolactin and ACTH stimulated the synthesis of estrogens by the tissue whereas GH, LH and ACTH were more effective than prolactin in stimulating androgen synthesis. The effect of protein hormones, other than ACTH, on adenylate cyclase activity of this tumour tissue indicated a lack of specificity of the membrane receptor sites.
In a longevity study a total of 394 Han:SPRD rats (200 males and 194 females) were kept in a barrier type animal quarter from weaning until their natural death. The mean life span of males was 29.6 +/- 5 months, that of females 27.6 +/- 6.2 months. The most frequent neoplasms in females were mammary gland tumours (63.4%). Neoplasms of endocrine organs were common in animals of both sexes. Thyroid neoplasms, predominantly of C-cell origin, occurred in 60% of the males and 51.5% of the females. Pituitary gland tumours were observed in 53% of male and 45.1% of female rats. Further frequent tumours of the endocrine system included phaeochromocytomas (23% in males, 15.5% in females), adrenal cortex neoplasms (6% in males, 13.5% in females) and tumours of islet cells of the pancreas (20.8% in males, 8.2% in females). An uncommonly high incidence of squamous cell neoplasms (16% in males; 9.3% in females) of the preputial and clitoral gland was observed in this stock. Further organs frequently affected by neoplasms were the skin/subcutis and brain.
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The monoclonal antibody D11 is a valuable aid in the accurate typing of adrenal tumours as, in formalin-fixed, paraffin-embedding material, strong nuclear D11 positivity was observed only in adrenocortical cells in 190 neoplasms (including 100 adrenal tumours). This pattern was demonstrated for all zona glomerulosa cells in 27 normal adrenals and for the neoplastic cells of 15 adrenocortical adenomas derived from that zone, as judged from clinically evident hyperaldosteronism. Normal cells of zona fasciculata and reticularis also showed strong diffuse D11 immunostaining and the same nuclear plus cytoplasmic D11 reactivity was evident in 15 benign and malignant adrenocortical neoplasms derived from these zones, documented by hypercortisolism. Cytoplasmic and/or nuclear D11 staining made topohistogenetic typing possible in 15 non-functioning cortical tumours. D11 immunostaining gave negative results in 50 specimens containing normal, hyperplastic and neoplastic adrenomedullary cells. In addition, absence of D11 reactivity was recorded in 4 adrenal metastases of extra-adrenal carcinomas, 5 paragangliomas, 25 primary renal carcinomas and 59 of 60 primary thyroid carcinomas. D11 immunocytochemistry allows the accurate typing of benign and malignant adrenocortical neoplasms, irrespective of histology and function. With this method, primary adrenocortical tumours can be separated from carcinomas metastatic to the adrenal gland, including secondary tumours of similar phenotype (such as renal carcinomas). By exclusion, D11 negativity provides evidence of the medullary origin of primary adrenal tumours even in the absence of clinical, structural, histochemical and conventional immunohistochemical indicators of phaeochromocytoma.
Pathological morphology, clinical features and nuclear DNA contents determined by flow cytometry (FCM) were studied in 11 cases of normal adrenals and 41 cases of adrenal neoplasms. It was found that despite differences in clinical manifestations and in degrees of cellular atypia of the adenomas their DNA contents were the same as in normal adrenals. On the other hand, the pheochromocytomas were benign in nature, but most of them (16/19) showed DNA aneuploidy and the DNA indices were correlated with their clinical and morphological features.
The clinical data and morphologic findings in 16 cases of adrenocortical carcinoma were compared with those in 11 cases of surgically removed functional adenomas and 12 cases of nonfunctional adenomas found at autopsy. Histopathologic changes of architectural disarray, pleomorphism, increased mitotic activity, vascular invasion, hemorrhage, or necrosis were generally reliable criteria for diagnosis of malignancy. However, weight was the parameter that most consistently correlated with outcome, since all patients with tumors under 50 Gm. survived and all lesions of 95 Gm. or over proved to be malignant.