Adrenal Gland Tumor
The adrenal gland is located on the superior portion of the kidney. It produces some of the most important hormones associated with body metabolism. Many metabolic pathways depend upon the adrenal gland functionality through its hormone production. Some of the significant hormones produced by the adrenal gland include aldosterone, cortisol, sex hormones and adrenaline. The adrenal gland comprises two distinctive regions cortex and medulla respectively. Each region of the adrenal gland produces different kinds of hormones.
Adrenal insufficiency is a disorder predominantly associated with the dysfunction of the adrenal cortex region. The intensity of this disorder can be represented as primary or secondary depending upon the impaired adrenal cortex and its functionality. Studies imply that autoimmune diseases such as Addison's disease are the predominant cause for the adrenal sufficiency. In the secondary condition of this disorder, the production of adrenocortico tropic hormone is reduced to a greater extent. Although autoimmune disorders play a major role in the adrenal insufficiencies, other causes such as infectious diseases, vascular impairments, congenital disorders and also iatrogenic causes lead to adrenal insufficiencies. These factors predominantly influence the production of glucocorticoids.
The adrenal insufficiencies associated with mineralocorticoids are caused because of underlying conditions such as corticosterone methyl oxidase deficiency, impairments in the Zona glomerulosa and treatments such as heparin therapy. The recommended treatment for adrenal insufficiency is usually through hormonal therapy. Care must be taken while administering hormones as it may induce side effects such as obesity. Hydrocortisone is administered to treat adrenal insufficiencies.
Adrenal Pheochromocytoma is a condition in which tumors are formed on the adrenal gland. These tumors occur predominantly on one adrenal gland as an unilateral condition. The bilateral condition is found only in rare cases. These tumors occur together with other disorders such as endocrine neoplasia type 2. Majority of these pheochromocytomas are benign in origin and only a few cases develop malignancy.
The symptoms of adrenal Pheochromocytoma include headaches, palpitation, profuse sweating, glucose intolerance, heat sensitivity and decreased blood pressure. Since pheochromocytomas are associated with variations in blood pressure, the diagnosis of the respective underlying condition is done by the determination of products obtained from the breakdown of adrenaline and noradrenaline. Other tests include the glucagon stimulation test for monitoring the blood pressure and the clonidine suppression test to detect the presence of tumors which lower the catecholamines. Confirmatory tests are done using radiological determination through CT scan and MRI scans.
Pheochromocytoma is treated with blood pressure lowering drugs such as alpha adrenergic blockers and the tumors associated are surgically removed. Post-operative care such as transfusion of fluids is given to patients who experience weakness because of low blood pressure.
Adrenal tumors are generally categorized into functional and nonfunctional depending upon their type. Tumors associated with the adrenal cortex fall into the functional category whereas the remaining part of the adrenal gland secreting mixed hormones such as sex hormones and aldosterone fall under the nonfunctional category. Metastases associated with adrenal gland are more prominent in the determination of preexisting neoplasms and underlying adrenal insufficiency. Adrenal tumors of the benign non-functional origin are also caused by other conditions such as amyloidosis and granuloma. The malignant nonfunctional tumors are caused by conditions such as neuroblastoma and lymphoma.
Adrenal tumors are identified by a careful examination of the patient history followed by biochemical and radiological investigations. Fine needle aspiration biopsy is advised for pathological determination of the existing tumor. Surgical procedures such as laparoscopic adrenalectomy have produced good results in the removal of adrenal tumors.
The ovaries produce estrogen, progesterone and androgens to regulate the menstrual cycle. When a hysterectomy occurs, these hormones get suddenly interrupted and their levels fall resulting in symptoms of menopause. This is termed surgical menopause. Although removal of ovaries becomes unavoidable in most hysterectomy surgeries, every effort is made by the surgeon to leave the ovaries intact in order to avoid the sudden absence of hormones. Most often, surgical menopause is caused quite dramatically when there is surgical interference like hysterectomy, bilateral oophorectomy, where both the ovaries are removed. A woman undergoing surgical menopause experiences certain symptoms more profoundly than women going through menopause normally. Since there is abrupt disruption of hormones after hysterectomy, the menopausal symptoms are more severe, more frequent and last longer when compared to natural menopause. The symptoms are triggered by the body's sudden inability to make certain hormones due to the removal of ovaries.
Estrogen is immediately given after surgery to try to prevent the intense changes especially the hot flashes that can occur in woman undergoing hysterectomy. However the use of estrogen is itself controversial and it is not usually recommended for women with existing or high risk of cardiovascular disease. A lowest dose of estrogen for the shortest possible time is recommended.
Surgical menopause risks
A rare hormonal condition characterized by delayed or absence of puberty and an impaired sense of smell is Kallmann syndrome. This belongs to a larger group of conditions known as hypogonadotrophic hypogonadism. This condition is usually characterized by a failure to start or fully complete puberty. However hypogonadotrophic hypogonadism can be treated successfully by means of specialized hormone replacement therapy.
It is estimated that this syndrome affects 1 in 10,000 to 86,000 people and occurs more often in males rather than in females. Kallmann Syndrome 1 is the most common form of this condition. It was Franz Kallmann, an American scientist who first published a paper in 1914 about this syndrome. Therefore this genetic condition is named after him.
Characteristics of Kallmann Syndrome
Males with hypogonadotrophic hypogonadism are born with unusually small penis and undescended testes. Whereas, females affected, usually do not begin menstruating at puberty and have little or no breast development at all. In some women, puberty is either incomplete or delayed. In short, at puberty those affected do not develop secondary sexual characteristics.
In Kallmann syndrome, the sense of smell is particularly diminished or completely absent. This feature indeed distinguishes this syndrome from most other forms of hypogonadotrophic hypogonadism.
The significant aspect here is that most patients are not aware of this inability to detect odors until they are diagnosed. Some points to remember:
Inability to smell strong warning smells such as smoke or gas.
Inability to smell caustic products such as bleach.
Inability to smell rotten/spoilt food.
Personal hygiene, body odor and clothes.
Since the sense of smell is also linked to the sense of taste, some foods will not taste the same as with other people.
Signs and Symptoms
Kallmann Syndrome, even within the same family may vary in its features. Some additional symptoms and signs may include failure of one kidney to develop, a cleft lip with or without an opening in the roof of the mouth or a cleft palate, abnormal eye movements, hearing loss and abnormalities in tooth development.
In some affected persons, the movements of one hand are mirrored by the other hand - Bimanual Synkinesis. This can make it difficult to undertake tasks that require the hands to move separately such as playing a musical instrument. Kallmann syndrome has types designated from 1 through 4 distinguished by their genetic cause. The four types are identified as four forms of Kallmann syndrome, each characterized by an impaired sense of smell. Additional features of cleft palate occur only in types 1 and 2.
Kallmann syndrome and other related conditions are congenital in nature and are present from birth. Their genetic basis may not be fully understood and this syndrome can be inherited through the generations although it is difficult for the doctor to predict if this will occur.
The genetic factor: Certain mutations in the KAL1, FGFR1, PROKR2, and PROK2 genes cause Kallmann syndrome. Mutations in FGFR1 gene causes syndrome type 2 and mutations in PROKR2 and PROK2 cause Kallmann syndromes types 3 and 4 respectively.
These genes seem to play a role in the development of the brain much before birth. They are involved in the formation and movement of a group of nerve cells that are specialized in olfactory or smell process. The mutations KAL1, FGFR1, PROKR2, and PROK2 genes which play a role in the migration of neurons that produce a hormone called GnRH – Gonodotropin releasing hormone that controls several other hormones that direct sexual development before birth and during puberty. These hormones are necessary for the normal functioning of the ovaries in women and testes in men. These mutations disrupt the olfactory nerve cells and GnRH producing nerve cells in the brain. However these conditions and features vary among individuals and thus additional genetic and environmental factors may be involved.
Without proper treatment, the affected female is likely to face infertility and have an increased risk of developing osteoporosis or brittle bones. However, with correct diagnosis and treatment, fertility can be achieved in many cases and risk of osteoporosis decreased.
Following clinical examination, certain biochemical parameters and various imaging tests are done to confirm the diagnosis. As this is a genetic condition, testing for various genetic forms of this disease may also assist in making the diagnosis.
Initially hormone replacement therapy - testosterone in males and estrogen and progesterone in females is used to help induce secondary sexual characteristics.
Once pubertal maturation happens, for fertility either injection of pituitary hormones – gonadotropins, LH and FSH and in some instances therapy with synthetic peptide, GnRH is given. Deficiency of these hormones causes syndromes, and these are required to induce the sex organs to make sperm in males and eggs in females.
In females, the steps taken may vary depending upon individuals and the desired outcome. In premenopausal women, a step-by-step increase in estrogen dosage is given. A bone scan is done to assess the bone age. Without the presence of estrogen, the bone age will be behind that normally seen at that chronological age. The aim is to match the estrogen dosage to the bone age such that the development of bone can be enhanced. The doctor usually monitors the stage of treatment very closely.
Specialist fertility treatments are available and these can induce a certain level of fertility. The aim is to produce hormones by the pituitary and hypothalamus glands and induce natural production of sex hormones. Results can take 6 to 18 months but it is possible to induce fertility in some cases.
There are various drugs available which are usually self-injected twice or thrice a week over a period of at least six weeks. To combat the risk of osteoporosis, it becomes necessary to take tablets that reduce the risk. These tablets help increase the calcium uptake by the bones to strengthen them. However, medication will not eliminate the risk of osteoporosis.
Early diagnosis, education, personal outlook and support from family and friends can help reduce the problems surrounding this syndrome. Kallmann syndrome is usually lifelong in nature and about 10-15% of patients may experience recovery of hormonal system.
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Diseases, Symptoms, Tests and Treatment arranged in alphabetical order:
Bibliography / Reference
Collection of Pages - Last revised Date: January 30, 2023