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    General And Endoscopy Surgery:
 
 

Adrenal Surgery:

The adrenal glands are golden-yellow triangle or crescent shaped glands which are attached to the top of each of your two kidneys. They are normally about 3 x 5 x 1 cm in size. Each gland had a cortex which surrounds the medulla. Each region of the gland is responsible for hormone production such as cortisol and aldosterone in the cortex and norepinephrine and epinephrine (adrenaline) in the medulla. The adrenal gland also manufactures some of the sex hormones.

WHEN IS ADRENAL SURGERY NEEDED?

Surgical removal of the adrenal gland (adrenalectomy) may be recommended in situations of hormone overproduction or if there is a concern that a mass of the adrenal gland may be a cancer.Three main situations exist where hormone overproduction is an indication to have an adrenalectomy. The first is Cushing's Syndrome in which excess steroid production (cortisol) is caused by a tumor of the adrenal cortex. The second is Conn's Syndrome in which a tumor of the same area produces excess aldosterone which may cause problems with high blood pressure and blood potassium levels. The third is a tumor of the medulla which is called a pheochromocytoma. This tumor may manufacture excess adrenaline and cause problems such as high blood pressure, excess sweating, tremor, and anxiety.

Sometimes an adrenal tumor is found incidentally during an ultrasound or CAT scan of the abdomen. Even if these tumors do not overproduce hormones and cause any of the problems above, adrenalectomy may be recommended due to the size or other characteristics of the mass. This is because of concern that the mass may be a cancer of the adrenal gland. Adrenal cancer is very rare, but adrenalectomy may be the only chance to cure the cancer. If the tumor is a cancer that has spread from another organ to the adrenal gland, that is not a good reason for adrenalectomy (with very few exceptions).


HOW IS ADRENAL SURGERY USUALLY PERFORMED?

Adrenalectomy may be performed in two main fashions - an open procedure or a laparoscopic procedure. Open operations may be performed through the back (sometimes requiring partial removal of a rib), the flank, or the abdomen. Laparoscopic procedures use small telescopes and long instruments to remove the adrenal gland through a series of small incisions. Typically, patients having laparoscopic procedures have less pain and a more rapid recovery. The method by which the adrenalectomy is performed depends on many factors including the specific disorder, tumor size, and general patient health. Laparoscopic adrenalectomy is not an appropriate operation if there is strong evidence that the tumor is an adrenal cancer.We have included a short video (1.4 mg / .mpg file) of an adrenalectomy for you to view. You may click here to begin.

Tumors of the adrenal cortex are reported in 2% of all autopsies, with the most common lesion being a benign adenoma. The common major pathologic entities of the adrenal gland that require surgical intervention are primary hyperaldosteronism (ie, Conn syndrome), Cushing syndrome, pheochromocytoma, neuroblastoma, and adrenocortical carcinoma. However, many adrenal glands are removed en bloc as part of a radical nephrectomy for renal cell carcinoma. Frequently, lesions metastatic to the adrenal gland necessitate adrenalectomy, and reports exist of adrenal excision for symptomatic adrenal cysts. The workup of adrenal disorders requiring surgical intervention has undergone a revolution with the tremendous advances in hormonal research, as well as in radiographic techniques and localization. In general, neoplastic lesions of the adrenal gland may be classified with the tumor, node, metastases (TNM) staging system.

Tumor

  • T1 - Tumor confined to adrenal gland and less than 5 cm
  • T2 - Tumor confined to adrenal gland and greater than 5 cm
  • T3 - Tumor invasion into periadrenal fat
  • T4 - Tumor invasion of adjacent organs

Node

  • N0 - Negative lymph nodes
  • N1 - Positive lymph nodes

Metastases

  • M0 - No metastases
  • M1 - Distant metastases

History of the Procedure: The adrenal gland is crucial to endocrine homeostasis, and maladies associated with it result in several recognized syndromes. Understanding of the adrenal glands began in 1805, when Currier first delineated the anatomic structure of the medulla and cortex. Addison later described the clinical effects of adrenal insufficiency in 1855. Thomas Addison first described the association of hypertensive episodes with adrenal tumors in 1886. Medical and surgical management of pheochromocytoma was first described in the United States by Mayo and remained relatively unchanged until the 1960s, when Crout elucidated the biochemical pathways and diagnostic catecholamine studies, allowing diagnostic ability prior to exploration.

Problem:

Primary hyperaldosteronism

First described in 1955 by Jerome Conn, the hallmarks of primary hyperaldosteronism are hypertension, hypokalemia, hypernatremia, and elevated urine aldosterone levels (with salt repletion), as well as decreased renin activity and alkalosis with increased urinary potassium excretion. Primary hyperaldosteronism can be secondary to an adrenal adenoma or secondary to bilateral adrenal hyperplasia. Differentiating between these two disease processes is important because they can be treated differently. The patient's renin level should also be checked to rule out causes of secondary hyperaldosteronism, such as renal artery stenosis. The renin level is elevated in persons with renal artery stenosis, while the renin level is suppressed in those with primary hyperaldosteronism.

Cushing syndrome

The diagnosis of Cushing syndrome is made based on abnormalities of urinary and plasma cortisol and/or adrenocorticotropic hormone (ACTH). The syndrome typically is attributed to central, hypothalamic, or pituitary excess secretion of ACTH (Cushing disease), primary adrenal hypercorticalism, or ectopic secretion of ACTH.

Pheochromocytoma

These tumors arise from chromaffin cells of the adrenal medulla. Ten percent of cases may be familial, and 10% might be bilateral or in extra-adrenal locations. If the tumor arises from a site other than the adrenal, it is termed a paraganglionoma. Paraganglionomas have been reported in locations from the neck to the pelvis. While pheochromocytoma follows the "rule of 10s," with only 10% of cases involving malignant tumors, 50% of cases of paraganglionomas have reported malignancies. Pheochromocytomas also can be a part of an endocrine syndrome such as multiple endocrine neoplasia (MEN) IIa, MEN IIb, von Hippel-Lindau disease, or von Recklinghausen disease.

Neuroblastoma

Neuroblastomas arise from sympathetic neuroblasts and occur almost exclusively in the pediatric population. Neuroblastoma represents the most common extracranial solid tumor in children, and approximately one third of neuroblastomas arise in the adrenal gland. Surgery of neuroblastoma is an important element in diagnosis, staging, and treatment of children with neuroblastoma. Surgery is curative therapy for patients with stage I and early stage II disease, with a reported 2-year survival rate of 89%. Reviews regarding safety reveal a low complication rate, commonly less than 10%. Advanced-stage tumors usually require a combination of surgery, chemotherapy, and/or radiation therapy to provide a complete response.

Adrenocortical carcinoma

Adrenocortical carcinoma is a rare disease with a poor prognosis. Up to 80% of adrenal carcinomas are functional and secrete multiple hormones. Frequency: In the United States, tumors of the adrenal cortex are reported in 2% of all autopsies, with the most common lesion being a benign adenoma. The incidence of adrenal carcinoma is estimated to be 1 case per 1.7 million, and it accounts for 0.02% of all cancers.

Etiology:

Primary hyperaldosteronism

The most common causes of aldosterone overproduction are idiopathic adrenal hyperplasia, followed by adenomas, and then (rarely) adrenal carcinoma. Of the benign adenomas, approximately 60% are unilateral (and typically managed surgically), while 40% are bilateral lesions.

Neuroblastoma

Neuroblastomas arise from sympathetic neuroblasts and occur almost exclusively in the pediatric population. Approximately one third of neuroblastomas arise in the adrenal gland.

Pathophysiology:

Primary hyperaldosteronism

The hallmarks of primary hyperaldosteronism are hypertension, hypokalemia, hypernatremia, and elevated urine aldosterone levels (with salt repletion), as well as decreased renin activity and alkalosis with increased urinary potassium excretion. The most common causes of aldosterone overproduction are idiopathic adrenal hyperplasia, followed by adenomas, and then (rarely) adrenal carcinoma. Of the benign adenomas, approximately 60% are unilateral (typically managed surgically), while 40% are bilateral lesions that are treated medically with spironolactone, unless marked asymmetry of aldosterone production is present. In this case, the dominant gland often is excised, unless bilateral disease that is uncontrollable by medical therapy exists.

Cushing syndrome

The syndrome typically is attributed to central, hypothalamic, or pituitary excess secretion of ACTH (Cushing disease), primary adrenal hypercorticalism, or ectopic secretion of ACTH.

Pheochromocytoma

These tumors arise from chromaffin cells of the adrenal medulla. Presentation of the pheochromocytoma varies with the production of active metabolites. Most commonly, episodic alpha-adrenergic hypersecretion leads to intermittent malignant hypertension.

Neuroblastoma

Neuroblastomas arise from sympathetic neuroblasts and occur almost exclusively in the pediatric population. Neuroblastoma represents the most common extracranial solid tumor in children, and approximately one third of neuroblastomas arise in the adrenal gland. They are rapidly growing tumors and may be metabolically active; however, the more common presentation is from mass effect.

Adrenocortical carcinoma

As the name implies, adrenocortical carcinoma arises from the cortex. The adrenal cortex in made up of 3 distinct zones: glomerulosa (outer), fasciculata (middle), and reticularis (inner). These 3 zones are responsible for aldosterone, cortisol, and sex steroid production, respectively. Up to 80% of adrenal carcinomas are functional and secrete multiple hormones. The most common hormones secreted are glucosteroids, followed by androgens, estradiol, and, finally, aldosterone. Adrenal carcinomas can be subclassified according to their ability to produce adrenal hormones.

Clinical:

Primary hyperaldosteronism

Presentation of primary hyperaldosteronism includes hypertension, hypokalemia, hypernatremia, and elevated urine aldosterone levels (with salt repletion), as well as decreased renin activity and alkalosis with increased urinary potassium excretion.

Cushing syndrome

The clinical presentation of Cushing syndrome is hypertension, moon facies, abdominal striae, buffalo hump, muscle weakness, amenorrhea, decreased libido, osteoporosis, fatigue, hirsutism, and obesity.

Pheochromocytoma

Presentation of the pheochromocytoma varies with the production of active metabolites. Pheochromocytoma most often develops in young–to–middle-aged adults. The classic triad is episodic headache, tachycardia, and diaphoresis. The most common clinical sign of pheochromocytoma is hypertension. Persons with this condition may experience sustained hypertension, paroxysmal hypertension, or sustained hypertension with superimposed paroxysms. Other common signs are palpitations, anxiety, tremulousness, chest pain, and nausea and vomiting. A small group of these patients experience induced myocardiopathy due to sustained catecholamine release. They present with decreased cardiac function and congestive heart failure. Generally, the cardiomyopathy is reversible with the use of antiadrenergic blocking agents and alpha-methylparatyrosine, a catecholamine synthesis inhibitor.

Neuroblastoma

Neuroblastomas arise from sympathetic neuroblasts and occur almost exclusively in the pediatric population. Neuroblastoma represents the most common extracranial solid tumor in children, and approximately one third of neuroblastomas arise in the adrenal gland. They are rapidly growing tumors and may be metabolically active; however, the more common presentation is from mass effect.

Adrenocortical carcinoma

These patients present with constitutional symptoms such as weight loss, fever, and malaise. Up to 80% of adrenocortical carcinomas are functional, and patients with these present with clinical signs of Cushing syndrome. An increase in sex steroid levels can result in oligomenorrhea, virilization, or feminization.The most common presentation of adrenocortical carcinoma is that of an incidentaloma. At presentation, 19% have inferior vena cava (IVC) involvement and 32% have metastases.

 

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