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Query: UMLS:C0027819 (neuroblastoma)
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Adrenal imaging using radiopharmaceuticals is a functional test that can contribute significantly to surgical management and follow-up of patients with either benign or malignant conditions of the adrenal cortex and medulla. Imaging of the cortex is achieved by iodine-131-labeled iodomethyl nor-cholesterol (NP-59), while adrenal medulla imaging can be successfully accomplished by 131I-metaiodobenzylguanidine (MIBG), which localizes in the adrenergic nerve terminal with norepinephrine. Both tests carry high sensitivity and specificity for functional tumors and hyperplasia, and often better than CT scanning. This article reviews the current status and clinical utility of nuclear imaging of the adrenal cortex in congenital hyperplasia, low renin hypertension and aldosteronism, and Cushing's syndrome. Adrenal medulla imaging is reviewed in light of our experience at the University of Texas M.D. Anderson Cancer Center in pheochromocytoma, neuroblastoma, and other neuroectodermal tumors. Investigation of 131I-MIBG therapy of metastatic tumors of neuroectodermal origin potentially offers a means of at least controlling symptoms of hormonal secretion in these patients.
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PMID:Role of adrenal imaging in surgical management. 217 29

Endocrine hypertension secondary to disorders of the adrenal glands is uncommon, but by no means rare. The importance of correct biochemical diagnosis and subsequent localization of the responsible lesion(s) lie in the fact that many of these syndromes occur in younger patients, may exhibit familial patterns of inheritance and are frequently amenable to surgical cure. The radiopharmaceuticals (131)1-6 beta-iodomethyl-19-norcholesterol (NP-59), a marker of adrenocortical cholesterol uptake, and (131)1- and (123)1-metaiodobenzylguanidine (MIBG), a norepinephrine (NE) analog and marker of energy-dependent NE storage vesicle accumulation, can be shown to accurately localize adrenal cortex and sympathoadrenal dysfunction, respectively. In Cushing's syndrome (CS) not only does the pattern of NP-59 uptake depict the adrenal dysfunction and its pathophysiologic basis, but the level of NP-59 accumulation reflects the degree of adrenocortical hyperfunction. Adrenocorticotrophin-independent CS is uniformly and accurately localized, especially in bilateral cortical nodular hyperplasia where even high resolution computed tomography (CT) may fail to depict the often subtle, asymmetric anatomic abnormalities. Dexamethasone suppression NP-59 adrenal scintigraphy has been shown to be highly sensitive and specific, and exceeds the efficacy of CT in the differentiation of adenoma and bilateral hyperplasia in primary aldosteronism. MIBG is useful as a sympathoadrenal imaging agent whose clinical utility has been demonstrated in the localization of pheochromocytoma, especially as a modality to screen the body for multiple and extraadrenal, recurrent, or metastatic lesions. Moreover, the extent of metastatic involvement from neuroblastoma can also be accurately depicted using MIBG. In this review we will examine the role of adrenal scintigraphy in the characterization of hypersecretory disorders of the adrenal cortex, medulla, and related conditions that produce hypertension as part of their symptom(s) complex. This approach, which is complementary to other anatomical modalities of imaging, can be used to advantage in the localization of functioning cortical and medulla adrenal diseases and other neoplasms of adrenergic origin.
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PMID:Scintigraphic studies in adrenal hypertension. 265 11

To study the relation of normetanephrine (NM) and metanephrine (M) to norepinephrine (NE) and epinephrine (E), plasma free NM (f-NM), free M (f-M), total NM (t-NM) and total M (t-M) were measured in normal subjects and patients with pheochromocytoma (PHEO), neuroblastoma, Cushing's syndrome, primary aldosteronism and chronic renal failure (CRF) by radioimmunoassay. Plasma f-NE and E were measured by radioenzymatic assay. Both f- and t-NM were high in PHEO, neuroblastoma and CRF. f- and t-M were also high in some patients with PHEO and CRF. Positive correlation was observed not only in f-NE with f-NM and t-NM, but also in f-E with f-M and t-M except for CRF. Although upright posture induced an elevation in f-NE and f-NM, t-NM was unchanged in normal subjects. In patients with PHEO, metoclopramide induced a prompt elevation in f-NE and E but no-change in t-NM and M levels. f-NE, f-E, f-NM, t-NM, f-M and t-M decreased rapidly after the resection of PHEO and reached the normal level on the third day after the surgery. In CRF patients, f-NM, t-NM, f-M and t-M decreased after hemodialysis despite an increase of f-NE. From these results, it was suggested that plasma NM and M levels reflected plasma NE and E to a certain extent in normal subjects and patients with normal renal function, and that the impaired renal function provoked an elevation of plasma NM and M due to the accumulation of them.
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PMID:[Plasma normetanephrine and metanephrine levels and their relationship to norepinephrine and epinephrine]. 322 Jan 54

Endocrine and neurological diseases are rare causes of arterial hypertension in childhood. They represent less than 5% of all cases of secondary hypertension. Inflammatory, traumatic, and tumorous disorders of the central nervous system rarely result in chronic hypertension but may frequently be associated with acute hypertensive crisis. The most important hypertensinogenic endocrine diseases are the catecholamine producing tumors pheochromocytoma and neuroblastoma and disorders of the adrenal cortex such as Cushing's syndrome, hyperaldosteronism, 11-hydroxylase deficiency and other mineralocorticoid excess syndromes. Renin producing tumors, hyperthyroidism and hyperparathyroidism are rare causes of hypertension in children. Neurogenic and endocrine forms of hypertension have contributed considerably to a better understanding of the pathophysiology of blood pressure regulation. They are of particular interest to the pediatrician since specific therapy may be available.
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PMID:[Endocrine and neurogenic hypertension in childhood]. 666 53

Adrenal disease can be manifested by endocrine dysfunction or anatomic abnormalities detected by cross-sectional imaging modalities. With the advent of newer and more reliable in vitro assays and a better understanding of the spectrum of adrenal pathology, the physician can now adopt a more accurate and cost-effective approach to the diagnosis of adrenal disease. Both functional and anatomic imaging modalities can play an important role in the evaluation of the incidental adrenal mass, the early detection of adrenal metastases, differentiation of the various causes of Cushings's syndrome, selection of patients for potentially curative surgery in primary aldosteronism and adrenal hyperandrogenism, and localization of pheochromocytomas and neuroblastomas. The usefulness of the adrenal cortical radiopharmaceutical, 131I-6-beta-iodomethylnorcholesterol (NP-59), and the adrenal medullary radiopharmaceuticals, 131I and 123I-metaiodobenzylguanidine (MIBG), is detailed for these various clinical settings and the role of NP-59 and MIBG is contrasted to that of the cross-sectional modalities, computed tomography and magnetic resonance imaging (MRI). Incidental adrenal masses are common, but malignancies are few. Imaging studies select those patients who require a further evaluation by biopsy examination or adrenalectomy. In the hyperfunctioning endocrine states, such as Cushing's syndrome, primary aldosteronism, adrenal androgenism, and pheochromocytoma, correlation of biochemical findings with both functional and anatomic imaging is necessary to avoid inappropriate and ineffective surgical intervention, yet not miss an opportunity for curative resection. Lastly, MIBG and MRI are complementary in the detection and staging of neuroblastoma.
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PMID:Adrenal cortical and medullary imaging. 757 43

Adrenal scintigraphy using 131I-6-beta-iodomethyl-19-norcholesterol or 6-methyl-75Se-methyl-19-norcholesterol is a function-dependent imaging method which, in association with high-resolution spatial imaging techniques, plays an essential role in the study of adrenocortical hyperfunction. It can distinguish between bilateral cortical hyperplasia and monolateral adenoma or carcinoma and can lateralise the adenoma. In patients with Cushing syndrome, in addition to allowing a distinction to be made between ACTH-dependent forms and independent forms, adrenocortical scintigraphy is particularly appropriate to identify non-common forms of adenomatous hyperplasia. Adrenocortical scintigraphy, performed during dexamethasone administration, is an accurate mean of differentiating bilateral adrenal hyperplasia from monolateral forms (adenoma or carcinoma) in patients with Conn's syndrome. Owing to the gradual spread of high-resolution spatial imaging techniques, the problem of the diagnostic classification of so-called "incidentalomas" (clinically silent masses discovered by chance) is a subject of considerable interest. Adreno-cortical scintigraphy appears to be able to provide an important contribution to identifying the functional behaviour of these tumours. Since the early 80s meta-iodobenzyl-guanidine (MIBG), marked with 131I or 123I, with a structure similar to norepinephrine and characterized by selective tropism for sympathetic and chromaffin tissue, has been used for the scintigraphic study of adrenal medulla. MIBG scintigraphy has been found to be particularly appropriate for the study of intra- and extra-adrenal, single and multiple, benign and malignant pheochromocytomas. This method has a high overall sensitivity and specificity. Lastly, MIBG scintigraphy is useful in the study of neuroblastoma.
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PMID:[Nuclear medicine methods for the diagnosis of adrenal tumors]. 765 Dec 80

A case of olfactory neuroblastoma in a 36-year-old woman who presented with florid Cushing's syndrome is reported. A nasal polyp, which proved to be an olfactory neuroblastoma, was resected. The procedure was followed by complete remission from the endocrinologic abnormalities. Postoperatively, the patient was well for 5 years until recurrence of both Cushing's syndrome and the nasal polyp was noted. Following combined transnasal-transcranial resection of the tumor, which extended into the anterior cranial fossa, the patient again experienced complete remission of Cushing's syndrome. Immunohistochemistry showed the tumor to be positive for neuron-specific enolase, synaptophysin, chromogranin, adrenocorticotropic hormone, beta-endorphin, and S-100 protein. Electron microscopy revealed neuritic processes containing microtubules and neurosecretory granules. This is the first reported case of Cushing's syndrome secondary to olfactory neuroblastoma.
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PMID:Cushing's syndrome secondary to olfactory neuroblastoma. 819 48

Since urine contains cross-reacting substances to anti-cortisol(F) antibody, we extracted F from urine samples with dichloromethane. Using this specific assay system for F with TDX dynapack(fluorescence polarization immunoassay system), clinical significance of urinary F was tested by measuring urine samples from normal subjects, patients with pituitary-adrenal diseases and liver cirrhosis, and pregnant. The circadian rhythm of urinary F was evaluated by using samples collected every 4-hour period. The amount of urinary excretions of F during 4 hours were the highest during between 4 a.m. to 8 a.m. and then gradually decreased reaching at the lowest level at between 0 a.m. and 4 a.m. in normal volunteers. Such circadian rhythm was not found in 6 patients with Cushing's syndrome. The urinary F levels in pregnant women started to increase in the samples from between 21 to 30 weeks of gestation, and the level was maintained even at the 5th day of post-partum. The urinary F levels in patients with liver cirrhosis were significantly lower than those for age-matched healthy volunteers. In patients with abnormal steroidogenesis, the urinary F levels in patients with Cushing's syndrome were extremely high, 165-3358 micrograms/day. On the other hands, those in patients with deficient steroid synthetase were 2.8-26.5 micrograms/day, and those in patients with neuroblastoma and pheochromocytoma were 7.8-43.8 micrograms/day. The urinary F levels were about 50-fold higher in the patients with Cushing's syndrome than those in the normal reference interval, whereas serum F and urinary 17-OHCS levels were only 2.5-fold and 7-fold higher than those healthy volunteers, respectively. These results indicate that the assay of urinary F extracted with dichloromethane is the most useful test for the diagnosis of Cushing's syndrome.
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PMID:[Evaluation of urinary excretion of free-cortisol in patients with abnormal pituitary-adrenal axes]. 908 34

The historical background at the University of Michigan laid a foundation for the innovative development of radionuclides in diagnosis and treatment of endocrine diseases. From that background, Dr. William Beierwaltes, the chief of Nuclear Medicine, inspired two talented young chemists to synthesize unique radiopharmaceuticals that transformed diagnostic approaches to certain endocrine disorders. Dr. Raymond Counsell's 131-I-radiocholesterol, enabled imaging that defined function in the adrenal cortex, and thereby distinguished the different forms of Cushing's syndrome and of primary aldosteronism; in addition, this new technique differentiated benign adrenal cortical adenomas from other adrenal cortical tumors. Dr. Donald Wieland created metaiodobenzlylguanidine (MIBG), a compound that can be tagged with either 131-I or 123-I, and led to the scintigraphic depiction of adrenergic tumors, particularly pheochromocytomas and neuroblastoma, anywhere in the body of a patient. Treatments with large doses of MIBG have reduced the malignant forms of pheochromocytomas and brought remissions to children with neuroblastomas. MIBG also concentrated in the autonomic neurons and so the nerves of the heart were also portrayed. Subsequent novel syntheses included positron-emitting nuclides that, through positron emission tomography, have revealed the physiology and altered physiology of the human heart. These men and their discoveries exemplify the creative endeavors that compel us to seek further the wonders of nuclear science.
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PMID:Radiopharmaceuticals for nuclear endocrinology at the University of Michigan. 1104 Oct 15

Laparoscopic adrenalectomy is considered to be the standard of care for the surgical excision of adrenal masses. The transperitoneal laparoscopic and retroperitoneoscopic approaches are described. Both are safe and as effective as open adrenalectomy, with the added benefit of the minimally invasive approach. It can be utilized for patients requiring surgery for a phaeochromocytoma, adrenal adenoma, adrenal adenocarcinoma, Cushing's syndrome, neuroblastoma, and an incidentaloma. Relative contraindications include previous surgery of the liver or kidney, large tumours (>8-10 cm in diameter) or coagulation disorders. Although the transperitoneal route is used more widely, the retroperitoneal approach provides direct access to the adrenal gland and easy visualization of the adrenal vein. It avoids also colonic mobilization, minimizes the risk of injury to hollow viscera, and the potential risk of adhesion formation. However, the reversed orientation of the kidney and hilum, combined with a significantly smaller working space, may make this approach difficult to master.
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PMID:Surgical technique: Retroperitoneoscopic approach for adrenal masses in children. 2444 Jun 95


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