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| {{Adrenal tumor}} | | {{Adrenal tumor}} |
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| ==Tumors of the Adrenal Cortex==
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| The [[adrenal cortex]] is composed of three distinct layers of [[endocrine]] cells which produce critical [[steroid hormone]]s. These include the [[glucocorticoid]]s which are critical for regulation of [[blood sugar]] and the [[immune system]], as well as response to physiological [[stress]], the [[mineralcorticoid]] [[aldosterone]], which regulates [[blood pressure]] and [[kidney]]function, and certain [[sex hormone]]s. Both [[benign]] and [[cancer|malignant]] tumors of the adrenal cortex may produce steroid hormones, with important clinical consequences.
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| ===Adrenocortical adenoma===
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| ''Adrenocortical adenomas'', or adrenocortical "nodules", are small, benign tumors of the adrenal cortex which are extremely common (present in 1-10% of persons at autopsy). The clinical significance of these neoplasms is twofold. First, they have been detected as[[incidental finding]]s with increasing frequency in recent years, due to the increasing use of [[CT scan]]s and [[magnetic resonance imaging]] in a variety of medical settings. This can result in expensive additional testing and invasive procedures to rule out the slight possibility of an early [[adrenocortical carcinoma]]. Second, a minority of adrenocortical adenomas are "functional", meaning that they produce [[glucocorticoid]]s, [[mineralcorticoid]]s, and/or [[sex hormone|sex steroids]], resulting in endocrine disorders such as [[Cushing's syndrome]], [[Conn's syndrome]] (hyperaldosteronism), [[virilization]] of females, or[[feminization (biology)|feminization]] of males. Functional adrenocortical adenomas are surgically curable.
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| ===Adrenocortical carcinoma===
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| :''Main article: [[Adrenocortical carcinoma]]
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| Adrenocortical carcinoma (ACC) is a rare, highly aggressive [[cancer]] of adrenal cortical cells, which may occur in children or adults. ACC's may be "functional", producing [[steroid hormone]]s and consequent [[endocrine]] dysfunction similar to that seen in many adrenocortical adenomas, but many are not. Due to their location deep in the [[retroperitoneum]], most adrenocortical carcinomas are not diagnosed until they have grown quite large. They frequently invade large vessels, such as the [[renal vein]] and[[inferior vena cava]], as well as [[metastasis|metastasizing]] via the [[lymphatics]] and through the [[blood]] to the [[lung]]s and other organs. The most effective treatment is [[surgery]], although this is not feasible for many patients, and the overall[[prognosis]] of the disease is poor. [[Chemotherapy]], [[radiation therapy]], and [[hormonal therapy (oncology)|hormonal therapy]]may also be employed in the treatment of this disease.
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| ==Tumors of the Adrenal Medulla==
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| The [[adrenal medulla]] is located anatomically at the center of each adrenal gland, and is composed of neuroendocrine (chromaffin) cells which produce and release [[epinephrine]] (adrenaline) into the bloodstream in response to activation of the [[sympathetic nervous system]]. [[Neuroblastoma]] and [[pheochromocytoma]] are the two most important tumors which arise from the adrenal medulla. Both tumors may also arise from extra-adrenal sites, specifically, in the [[paraganglia]] of the [[sympathetic chain]].
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| ===Neuroblastoma===
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| :''Main article: [[Neuroblastoma]]
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| Neuroblastoma is an aggressive [[cancer]] of immature neuroblastic cells (precursors of [[neuron]]s), and is one of the most common[[pediatrics|pediatric]] cancers, with a median age at diagnosis of two years. Adrenal neuroblastoma typically presents with a rapidly enlarging abdominal mass. Although the tumor has often spread to distant parts of the body at the time of diagnosis, this cancer is unusual in that many cases are highly curable when the spread is limited to the [[liver]], [[skin]], and/or [[bone marrow]] (stage IVS). Related, but less aggressive tumors composed of more mature neural cells include ''ganglioneuroblastoma'' and''ganglioneuroma''. Neuroblastic tumors often produce elevated levels of [[catecholamine]] hormone precursors, such as[[vanillylmandelic acid]] (VMA) and [[homovanillic acid]], and may produce severe watery [[diarrhea]] through production of[[vasoactive intestinal peptide]]. Treatment of neuroblastoma includes [[surgery]] and [[radiation therapy]] for localized disease, and [[chemotherapy]] for [[metastasis|metastatic]] disease.
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| ===Pheochromocytoma===
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| :''Main article: [[Pheochromocytoma]]
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| Pheochromocytoma is a [[neoplasia|neoplasm]] composed of cells similar to the [[chromaffin cell]]s of the mature adrenal medulla. Pheochromocytomas occur in patients of all ages, and may be sporadic, or associated with a hereditary cancer syndrome, such as[[multiple endocrine neoplasia]] (MEN) types IIA and IIB, [[neurofibromatosis]] type I, or [[von Hippel-Lindau syndrome]]. Only 10% of adrenal pheochromocytomas are [[cancer|malignant]], while the rest are [[benign|benign tumors]]. The most clinically important feature of pheochromocytomas is their tendency to produce large amounts of the [[catecholamine]] hormones [[epinephrine]](adrenaline) and [[norepinephrine]]. This may lead to potentially life-threatening [[high blood pressure]], or cardiac[[arrythmia]]s, and numerous symptoms such as [[headache]], [[palpitation]]s, [[anxiety attack]]s, [[sweat|sweating]], [[weight loss]], and [[tremor]]. Diagnosis is most easily confirmed through urinary measurement of catecholamine [[metabolite]]s such as[[vanillylmandelic acid|VMA]] and [[metanephrine]]s. Most pheochromocytomas are initially treated with anti-adrenergic drugs to protect against catecholamine overload, with surgery employed to remove the tumor once the patient is medically stable.
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| ==References== | | ==References== |
| | {{Reflist|2}} |
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| {{reflist|2}} | | {{WH}} |
| {{Epithelial neoplasms}} | | {{WS}} |
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| | [[Category:Needs content]] |
| [[Category:Disease]] | | [[Category:Disease]] |
| | [[Category:Endocrine system]] |
| | [[Category:Endocrinology]] |
| | [[Category:Overview complete]] |
| | [[Category:Up-To-Date]] |
| [[Category:Oncology]] | | [[Category:Oncology]] |
| [[Category:Endocrine system]] | | [[Category:Medicine]] |
| [[Category:Endocrinology]] | | [[Category:Endocrinology]] |
| | | [[Category:Surgery]] |
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