[Bilateral total adrenalectomy in hypophyseal Cushing's syndrome and its results].
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Administration, Oral↗
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Results for “ADRENAL GLAND NEOPLASMS”
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Renin-angiotensin system.
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Adrenal Cortex↗
Adrenal masses in patients with malignancy: prospective comparison of echo-planar, fast spin-echo, and chemical shift MR imaging.
PURPOSE: To assess echo-planar, fast spin-echo (SE), and chemical shift magnetic resonance (MR) imaging in differentiation of adrenal adenomas from malignant adrenal masses in patients with known malignancy. MATERIALS AND METHODS: Sixty-eight adrenal masses (23 malignant, 45 benign) in 68 patients with known malignancy were examined with echo-planar, fast SE with and without fat suppression, and chemical shift pulse sequences. RESULTS: With a cutoff T2 value of 75 msec, the sensitivity of echo-planar imaging for benign lesions was 82%, and specificity was 96%. With a cutoff adrenal mass-to-spleen signal intensity ratio of 0.80, the sensitivity of fast SE imaging for benign lesions was 53%, and specificity was 96%. With a cutoff adrenal mass-to-spleen ratio of 0.55, the sensitivity of chemical shift imaging for benign lesions was 80], and specificity was 100%. CONCLUSION: Chemical shift imaging and calculated T2 values from echo-planar imaging are promising techniques for differentiation of adrenal adenomas from malignant adrenal masses and can obviate biopsy.
Adenoma↗
Percutaneous fine-needle aspiration biopsy cytology of the kidney and adrenal.
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Adenoma↗
[Hippel-Lindau syndrome and sporadic renal cell carcinomas. Pathogenesis, morphologic spectrum and molecular genetics].
von Hippel-Lindau (VHL) disease is an autosomal dominant heritable disease that often occurs in association with various benign and malignant tumours. Clinically the disease is classified as VHL 1 (without phaeochromocytoma) and VHL 2 (with phaeochromocytoma). Genetically, VHL is caused by germline mutations in the VHL tumour suppressor gene. More than 100 germline mutations and rearrangements have been identified, but the biological function of a hypothetical VHL protein is not yet known. Genotype-phenotype correlations should aid in the understanding of this biological role. All VHL manifestations subsequently develop to VHL mutations, but some mutations may act in a tissue-specific manner. Whereas missense mutations cause tumour suppression to fail in adrenal cells, more severe structural mutations are usually necessary for tumour development in renal cells. As predicted by the tumour suppressor theory, the VHL gene also plays a critical part in the pathogenesis of sporadic non-VHL-associated tumours. In a large number of sporadic renal clear cell carcinomas, mutations and hypermethylation cause inactivation of the VHL gene. Together with allelic 3p loss, these constitute rate-limiting events in renal tumourigenesis. Insights into the molecular basis of phenotypic variability in VHL disease and the confirmation of the tumour-suppressor criteria in VHL and non-VHL sporadic tumours indicate an important role of the VHL gene in the development of these tumours.
Adrenal Gland Neoplasms↗
Laparoscopic ultrasound.
Laparoscopic ultrasonography is a relative latecomer to the area of surgical sonography whose arrival can be attributed to the need for development of specialized transducers that could fit through conventional laparoscopic trocars. The quality, reliability, and ease of use of such units has evolved rapidly, so that laparoscopic ultrasonography may now be performed on a routine basis. Laparoscopic ultrasonography allows the surgeon to look into the tissues being operated upon, thereby compensating for the inability to physically palpate such tissues. Thus, it has not only helped to mimic open surgery, but has also refined the current techniques of laparoscopic operations. With the increasing availability of equipment, as well as the training of surgeons in this modality, laparoscopic ultrasound is quickly becoming an essential tool for the surgeon aiming to take laparoscopic surgery to new frontiers.
Adenoma↗
Laparoscopic surgery for pheochromocytoma: adrenalectomy, partial resection, excision of paragangliomas.
PURPOSE: Surgical treatment of pheochromocytoma includes adrenalectomy, adrenal sparing surgery and excision of extraadrenal paragangliomas. We report our experience using laparoscopy for these procedures. MATERIALS AND METHODS: Between June 1992 and November 1997, 19 patients underwent laparoscopic surgery for pheochromocytoma. Of the patients 14 had solitary tumors, and 4 presented with bilateral pheochromocytomas and 1 or 2 additional paragangliomas. In 1 patient a recurrent pheochromocytoma was found in the contralateral adrenal following previous right adrenalectomy. One patient each had myocardiopathy, amaurosis and stroke secondary to severe hypertension. Two patients were pregnant. RESULTS: All solitary tumors were treated with laparoscopic adrenalectomy. Laparoscopic adrenal sparing surgery (4 cases, 2 bilateral) and bilateral adrenalectomy (1) were performed for multiple familial pheochromocytoma, and all paragangliomas were excised simultaneously. The pregnant patients underwent surgery at 16 and 20 weeks of gestation, respectively. All procedures were completed as planned. The rate of minor intraoperative and postoperative complications was 11% and 16%, respectively, and there were no major complications. In all patients the catecholamine levels returned to normal and no residual tumors were found at followup. None of the patients undergoing partial resection required steroid replacement therapy. CONCLUSIONS: In experienced hands, laparoscopic surgery for solitary and multiple pheochromocytoma and paraganglioma is feasible and safe, and does not increase the specific risks associated with pheochromocytoma surgery.
Adrenal Gland Neoplasms↗
Diagnosis of adrenal tumors with radionuclide imaging.
The development of radiolabeled cholesterols in 1969 as precursors of adrenocortical steroid production allowed the first noninvasive imaging of the adrenal cortices. FDA-NDA approval in 1984 should allow routine use of these agents in most hospitals. NP-59 is most commonly used in the diagnosis and management of Cushing syndrome; the second most common use is in the diagnosis of primary aldosteronism. It is also helpful in the differential diagnosis of adrenal and ovarian hyperandrogenism and hirsutism, and is the only noninvasive method of detecting unilateral adrenocortical hypofunction. The newest and most popular use is in the differential diagnosis of asymptomatic masses in the region of the adrenal gland discovered incidentally with CT scan ("incidentalomas"). In this situation, the NP-59 scan can define whether the tumor is in the adrenal gland and if it is functional or nonfunctional. We believe that, in the future, radiolabeled enzyme inhibitors might offer better diagnostic imaging of the adrenal cortex, although these agents will probably not be available for routine use for some time. Our development of a radioiodinated guanethidine analog, 131I-MIBG, has allowed differentiation of normal adrenal medullary function from bilateral adrenal medullary hyperplasia before the development of hypertension or tachycardia, diagnostic increases in plasma or urinary catecholamines, or abnormal CT scans. The search for a pheochromocytoma should begin with 131I-MIBG scintigraphy. While over 90% of primary pheochromocytomas occur in the abdomen, neither a survey of the abdomen nor the finding of a single tumor should conclude the search. Whereas the CT scan can only detect a mass, the MIBG scan not only detects the mass but proves whether the mass is a pheochromocytoma. We have detected small or recurrent pheos when all other localizing studies were normal. Present drug and X-ray therapy is relatively ineffective in treating metastatic cancer of the adrenal medulla. Preliminary results in five patients indicate that, for the first time, a complex organic molecule can carry 131I into adrenal neoplasms reproducibly and in therapeutically effective doses of irradiation. We may also be able to select which patients will respond to therapeutic doses of 131I-MIBG and which patients will not respond.
Adenoma↗
Corticomedullary tumors of the adrenal glands. Report of two cases. Association of corticomedullary tumor with spindle cell sarcoma.
We describe two cases of corticomedullary tumors of the adrenal gland. The patients suffered from Cushings syndrome and paroxysmal hypertension. The corticomedullary tumors consisted of benign looking cortical adenoma cells growing on the background of the pheochromocytoma cells. We further present the ultrastructural and immunohistochemical features of these tumors. Focally a spindle cell sarcoma arising from the corticomedullary tumor was found in one case. The spindle cell sarcoma was immunohistochemically negative with antibodies to chromogranin, synaptophysin, cytokeratin and S-100 protein. Ultrastructurally the sarcoma was composed of undifferentiated primitive cells poorly endowed with cytoplasmic organelles. Focal transitions of the pheochromocytoma into the spindle cell sarcoma were seen. It is hypothesized that the spindle cell sarcoma was arising from the pheochromocytoma component of the corticomedullary tumor.
Adrenal Cortex Neoplasms↗
NF-1C, Sp1, and Sp3 are essential for transcription of the human gene for P450c17 (steroid 17alpha-hydroxylase/17,20 lyase) in human adrenal NCI-H295A cells.
Cytochrome P450c17 catalyzes steroid 17alpha-hydroxylase and 17,20 lyase activities, which are required for the biosynthesis of cortisol and sex steroids. Human P450c17 is expressed in a cAMP-responsive, cell-specific, developmentally programmed fashion, but little is known about its transcriptional regulation. Expression of deletion mutants of up to 2,500 bp of human 5'-flanking DNA in human adrenal NCI-H295A cells indicated that most regulatory activity was confined to the first 227 bp. Deoxyribonuclease I footprinting of the proximal promoter identified the TATA box, an steroidogenic factor-1 site, and three previously uncharacterized sites at -107/85, at -178/-152, and at -220/-185. EMSAs and methylation interference assays suggested that the -107/-85 site and the -178/-152 site bind members of the NF-1 (nuclear factor-1) family of transcription factors. An NF-1 consensus sequence generated similar DNA/protein complexes, and antibodies against NF-1C2/CTF2 supershifted the complexes formed by the -107/-85 site, the -178/-152 site, and the NF-1 consensus site. Western blots of nuclear extracts from NCI-H295A cells probed with this NF-1 antiserum identified two NF-1 isoforms between 50 and 55 kDa. The presence of NF-1C2 (CTF2) and CTF5 in NCI-H295A cells was demonstrated by RT-PCR and sequencing. Mutation of both the -107/-85 and the -178/-152 NF-1 sites reduced basal transcription by half. Supershift assays showed that the ubiquitous proteins Sp1 and Sp3 both bind to the -227/-184 region, and that mutation of their binding sites reduced transcription by 75%. Mutation of the Sp1/Sp3 site plus the two NF-1 sites eliminated almost all detectable transcription. Thus, Sp1 and Sp3 binding to the -227/-184 site and NF-1C proteins binding to the -107/-85 and the -178/-152 sites are crucial for adrenal transcription of the human gene for P450c17.
Adrenal Gland Neoplasms↗
Characterization of immunoreactive corticotropin and corticotropin-releasing factor in human adrenal and ovarian tumours.
The distribution of immunoreactive ACTH (I-ACTH) and corticotropin-releasing factor (I-CRF) in the human adrenal was determined and these peptides were indentified in adrenocortical adenomas from patients with primary aldosteronism. Cushing's syndrome, and ovarian tumours and compared with the results in phaeochromocytomas. I-ACTH and I-CRF, mainly localized in the adrenal medulla from patients without endocrine disorders, showed a good correlation with the epinephrine concentrations. I-ACTH and I-CRF were present in all the above-mentioned tumours. Gel filtration of I-ACTH and I-CRF from these tumours showed the presence of large molecular weight forms of these peptides as well as authentic peptides. The trypsinization study of I-CRF from phaeochromocytoma suggested that such large molecular weight forms were the precursors of authentic CRF. High performance liquid chromatography showed I-CRF in these tumours to be similar to hypothalamic CRF. Phaeochromocytoma tissue slices incorporated [3H]leucine into ACTH and CRF. These findings raised the possibility that I-ACTH and I-CRF are synthesized and processed in phaeochromocytoma.
Adenoma↗
Morphology and function of human neuroblastoma xenotransplanted in nude mice.
Nine human neuroblastomas were serially transplanted in nude mice. All transplanted tumors were taken from patients older than the age of 1 year 10 months. In contrast, the authors were unable to transplant tumors from patients younger than 1 year 7 months of age, in spite of many attempts. All transplanted tumors produced varying amounts of cathecolamines as revealed by the liquid chromatography and formalin-induced fluorescence techniques. The content of the cathecolamines was closely correlated with the number and size of secretory granules. In high cathecolamine-producing tumors, cell clusters showing strong yellow-orange fluorescence plus a positive argyrophil reaction suggesting pheochromoblastic differentiation were demonstrated by our combined cathecolamine fluorescence and argyrophil reaction method. One of the transplantable tumor lines was demonstrated by unique host responses and biochemical data, to produce adrenocorticotropic hormone and increased cathecolamines. Our observations indicate that neuroblastoma derived from neural crest cells seem to have a nearly complete hormone production function as compared with their normal counterparts.
Adrenal Gland Neoplasms↗
Immunohistochemical study of heat shock proteins 27, 60 and 70 in the normal human adrenal and in adrenal tumors with suppressed ACTH production.
Heat shock proteins (HSPs) are known to protect cells against various aggressions and to assist in the correct folding of nascent proteins as well as in the recovery of denatured ones. HSP70 increases its levels in the cell in response to any stress and is induced by ACTH in the adrenal gland. HSP60 is located in the mitochondria and assists in the folding of mitochondrial peptides. HSP27 is the only small HSP that is stress-induced. HSP27 and HSP70 are known to protect cells against apoptosis while, on the contrary, HSP60 is proapoptotic, increasing caspases maturation. We studied the expression of these HSPs in human adrenal tissue both in the normal glands (12 cases) and in tumoral tissue from cortisol producing adrenal adenomas (6 cases). Besides being neoplastic, these cells live in a particular ambience of lack of ACTH due to the suppression of the hypothalamic-pituitary ACTH secretion induced by the elevated levels of cortisol. HSP27 is highly expressed in the normal adrenal and shows a marked reduction of expression in Cushing's adrenal tissue. Although with overall lower levels of expression in the normal adrenal, HSP70 exhibited a similar pattern of reduction in tumoral tissue. HSP60, on the other hand, increased significantly and consistently in adrenal Cushing tumors. Besides the possible consequences of incorrect folding of nascent peptides, the alterations observed in tumoral tissue seem to act in an apoptotic direction. The only factor that we observed that could be contributing to these changes was the lack of plasma ACTH.
Adrenal Gland Neoplasms↗
Technical difficulties of selective venous blood sampling in the differential diagnosis of female hyperandrogenism.
To determine glandular steroid release of adrenals and ovaries in female hyperandrogenism, a standardized method for percutaneous transfemoral venous blood sampling was developed. In eight volunteers and 67 patients, catheterization was performed during the early follicular phase (days 3-7; between 8 and 10 a.m.) to reduce interference from cyclic and circadian variations of secretion. Serial samplings reduced the episodic effluent changes. Anatomical variations and collateral flow as well as stress effects and the dosage of contrast media were studied. During catheterization, peripheral cortisol levels did not differ significantly from control groups. Collaterals had no effect on hormone levels. Contrast media increased cortisol effluent levels only when they were sampled following venography. Four-vessel venous sampling was found to be indicated if peripheral testosterone was more than 1.5 ng/ml and/or dehydroepiandrosterone sulfate more than 6,700 ng/ml. If an ovarian (adrenal)/peripheral gradient of testosterone exceeded 2.7 ng/ml, surgical intervention for tumor removal at the site of hormone excess was felt to be necessary.
Adolescent↗
Minor morphologic abnormalities of adrenal glands at CT: prognostic importance in patients with lung cancer.
PURPOSE: To determine the prognostic importance of minor morphologic abnormalities of the adrenal glands at computed tomography (CT) in patients with lung cancer. MATERIALS AND METHODS: The study was approved by the committee on human research; written informed consent was not required. The authors retrospectively identified 197 patients with lung cancer who underwent serial chest or abdominal CT and did not have a focal adrenal mass at baseline CT. Two readers independently classified the morphologic features of each adrenal gland as normal, smoothly enlarged, or nodular at initial CT examination. They separately recorded the presence or absence of metastases to the adrenal glands (ie, any new focal adrenal mass) at final CT examination; a third independent reader arbitrated when interpretations were discordant (n = 11). Multivariate Cox proportional hazard models were used to assess for associations between baseline adrenal gland morphologic features and subsequent development of adrenal metastases. RESULTS: At initial CT, reader 1 classified 253 (64%), 70 (18%), and 71 (18%) of the 394 adrenal glands and reader 2 classified 258 (65%), 45 (11%), and 91 (23%) of these glands as normal, smoothly enlarged, or nodular, respectively. The readers had moderate interobserver agreement regarding the classification of adrenal gland morphologic features (kappa = 0.54). Metastases subsequently developed in 13 adrenal glands in 11 patients. Cox proportional hazard models revealed no significant association between baseline adrenal gland morphologic features and subsequent development of adrenal metastases (P = .50 and P = .20 for readers 1 and 2, respectively). CONCLUSION: In patients with lung cancer, smooth enlargement or nodularity of the adrenal glands at baseline CT is not associated with increased risk of subsequently developing adrenal metastases.
Adrenal Gland Neoplasms↗
Malignant pheochromocytoma associated with germline mutation of the SDHB gene.
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Adrenal Gland Neoplasms↗
Multiple endocrine neoplasia 2A syndrome presenting as peripartum cardiomyopathy due to catecholamine excess.
We report the case of a 24-year-old female with a history of medullary thyroid carcinoma who presented at 38 weeks gestation with acute chest pain and shortness of breath. She was found to be in pulmonary edema and respiratory failure. An emergency cesarean section was performed. Subsequently, an echocardiogram revealed an ejection fraction of 10%. After medical therapy with digoxin, milrinone, captopril and diuretics, her condition improved rapidly and a repeat echocardiogram showed that the left ventricular function had normalized. Diagnosis of pheochromocytoma was made by urine and plasma catecholamine measurements. Magnetic resonance imaging revealed a 3.7 cm left adrenal mass. Increased uptake activity was seen in the same region by an (131)I-metaiodobenzylguanidine (MIBG) scan. The patient underwent successful surgical resection of the pheochromocytoma. Subsequent DNA analysis revealed that the patient had a mutation of the RET proto-oncogene. The same mutation was also found in several of her family members. In summary, we report a case of multiple endocrine neoplasia 2A presenting as peripartum cardiomyopathy and cardiovascular collapse. Pheochromocytoma should be considered as a potential cause of peripartum cardiomyopathy.
3-Iodobenzylguanidine↗
[Uptake of 123I-MIBG in a hepatic hemangioma in the scintigraphic study of an adrenal gland lesion].
A 60 year old symptom free female in whom a lesion in left adrenal gland was found by chance in a CT scan is presented. She also had increased serum and urine catecholamines levels. 123I-MIBG scintigraphy showed a non-physiological uptake in right adrenal gland that is still seen in the delayed image, with normal left gland. MRI confirmed the presence of a mass in the left adrenal gland suggestive of an adenoma and found a lesion in the right hepatic area at the level of the previously seen MIBG image. This lesion was labelled as a hemangioma and would explain the findings of the isotopic study with MIBG. It must be considered as a false positive for phaechromocytoma. The increased catecholamine serum and urine levels were due to drug interactions.
3-Iodobenzylguanidine↗