SISTEM ENDOKRIN

PATOLOGI ANATOMIFK UNIMAL

BANDAR LAMPUNG

NORMAL

Manifestasi Kelainan Hipofisis

Hyperpituitarism Hypoppituitarism Efek masa lokal Penyakit hipofisis posterior

Pituitary Adenomas and Hyperpituitarism

functional silent Pituitary adenomas are classified

on the basis of hormone(s) produced by the neoplastic cells detected by immunohistochemical stains performed on tissue sections

Pituitary Adenomas and Hyperpituitarism

PROLACTINOMAS

Prolactinoma with hyperprolactinemia - is most common/30% pituitary tumor - staining chromophobe - in women amenorrhea & galactorrhea - caused by hypothalamic lesions or medications methyl dopa, reserpine interfere with dopamine (prolactin-inhibitory factors) secretion - can also be associated with estrogen therapy

GROWTH HORMONE (SOMATOTROPH CELL) ADENOMAS Somatotropic adenoma with hypersecretion of growth hormone - 2nd most common pituitary tumor - staining acidophyl - causes secondary hyperfunction of somatomedins by the liver. End organ effects are caused by both growth hormone and somatomedins, especially somatomedin C (insulin-like growth factor 1/IGF-1) - results gigantism if adenoma develops before epiphyseal closure and acromegaly if adenoma develops after epiphyseal closure - acromegaly overgrowth of jaws, face, hands and feet, and general enlargement of viscera with hyperglycemia, osteoporosis and hypertension - can also result in local compression effects due to expansion of the tumor within the sella tursica

CORTICOTROPH CELL ADENOMAS

Corticotropic adenoma and hypersecretion of ACTH - results in increased production of ACTH / hypercorticism - is called Cushing syndrome or Cushing disease 1. Cushing disease hypercorticism adenoma most often a basophilic adenoma basophilic microadenoma 2. Cushing syndrome hypercorticism regardless of cause, is most often of pituitary and less often of adrenal origin may be due to ectopic ACTH production by various tumors especially small cell carcinoma of lung

Hypopituitarism

Tumors and other mass lesions Pituitary surgery or radiation Ischemic necrosis of the pituitary

and Sheehan syndrome Rathke cleft cyst Empty sella syndrome Genetic defects

Selective deficiency of one or more pituitary hormones (1) deficiency of growth hormone - in children, result in growth retardation (pituitary dwarfism) - in adults, may result in increased insulin sensitivity with hypocalcemia, decreased muscle strength and anemia (2) deficiency of gonadotropins - in preadolescent children, results in retarded sexual maturation - in adults, results in loss of libido, impotence, loss of muscular mass, and decreased hair in men, and amenorrhea and vaginal atrophy in women (3) deficiency of TSH - result in secondary hypothyroidism (4) deficiency of ACTH - results in secondary adrenal failure - does not result in hyperpigmentation of the skin, probably because of lack of both ACTH and -MSH; this is in contrast to primary adrenal failure (Addison disease), in which ACTH is increased and hyperpigmentation is the rule

POSTERIOR HYPOPHYSIS (NEUROHYPOPHYSIS) HORMONES - are synthesized in the hypothalamus and transported via axons to the posterior pituitary a. Oxytocin : induces uterine contarction during labor and ejection of milk from mammary alveoli b. Anti diuretic hormone (ADH, vasopressin) - promotes water retention through

action on the renal collecting ducts

Syndrome of inappropriate ADH(SIADH) secretion is most commonly caused by ectopic production of ADH by various tumors, especially small cell carcinoma of lung. Results in retention of water with consequent dilutional hyponatremia, reduced serum osmolality, and inability to dilute urine Deficiency of ADH: results in diabetes insipidus; characterized by polyuria, with consequent dehydration and insatiable thirst - can be caused by tumors, trauma, inflammatory processes, lipid storage disorders, and other conditions characterized by damage of the neurohypophysis or hypothalamus

C. NON FUNCTIONING PITUITARY TUMORS

Non secreting pituitary adenomas - are most often chromophobe - result in dysfunction because of local pressure phenomena - are clinically variable ;manifestations include hypopituitarism, headache,visual disturbance (bilateral hemianopsia / loss of peripheral visual fields due to pressure on optic chiasm), and palsies caused by cranial nerve damage

Craniopharyngioma - is benign childhood tumor derived from remnants of the Rathke pouch - is not a true pituitary tumors - similar to ameloblastoma of the jaw - is characterized by nests and cords of squamous or

columnar cells in loose stroma, closely resembling the appearance of the embryonic tooth bud enamel organ

- is often cystic; lining epithelium of flat or columnar cells often expands into papillary projections

- is often detected radiographically because of calcification

THYROID

Hyperthyroidism Thyrotoxicosis is a hypermetabolic state caused

by elevated circulating levels of free T3 and T4 The three most common causes of thyrotoxicosis

are also associated with hyperfunction of the gland and include the following:   •    Diffuse hyperplasia of the thyroid associated

with Graves disease (accounts for 85% of cases)

  •    Hyperfunctional multinodular goiter   •    Hyperfunctional adenoma of the thyroid

Prolonged hypersecretion of thyroid hormone can result from (1) abnormal thyroid stimulator (Graves

disease), (2) intrinsic disease of the thyroid gland

(toxic multinodular goiter or functional adenoma), and

(3) excess TSH production by a pituitary adenoma (rare).

Pathogenesis

IMMUNE MECHANISMS IgG antibodies that bind to the TSH

receptor on the plasma membrane of thyrocytes stimulate the TSH receptor activating adenylyl cyclase and increasing thyroid hormone secretion thyroid becomes diffusely hyperplastic and excessively vascular

GENETIC FACTORS

GENDER EMOTIONAL

INFLUENCES SMOKING OPHTHALMOPATH

Y

Major clinical manifestations of Graves disease

Hypothyroidism Hypothyroidism refers to the clinical

manifestations of thyroid hormone deficiency It can be the consequence of three general

processes: Defective thyroid hormone synthesis, with

compensatory goitrogenesis (goitrous hypothyroidism) Inadequate thyroid parenchyma function, usually due

to thyroiditis, surgical resection of the gland, or therapeutic administration of radioiodine

Inadequate secretion of TSH by the pituitary or of thyroid-releasing hormone (TRH) by the hypothalamus

Primary (Idiopathic) Hypothyroidism is Often Autoimmune antibodies that block TSH or TSH receptors

without activating the thyroid Goitrous Hypothyroidism Reflects

Inadequate Secretion of Thyroid Hormone

The etiology of goitrous hypothyroidism includes iodine deficiency, antithyroid agents (drugs or dietary goitrogens), long-term iodide intake, and a number of hereditary defects in thyroid hormone synthesis

Endemic Goiter Endemic goiter is goitrous

hypothyroidism due to dietary iodine deficiency in locales with a high prevalence of the disease

Congenital Hypothyroidism is also Termed Cretinism

Dominant clinical manifestations of hypothyroidism

Thyroiditis

Thyroiditis describes a heterogeneous group of inflammatory disorders of the thyroid gland, including those that are caused by autoimmune mechanisms and infectious agents.

HASHIMOTO THYROIDITIS

SUBACUTE (GRANULOMATOUS) THYROIDITIS

caused by a viral infection or a postviral inflammatory process

majority of patients have a history of an upper respiratory infection just before the onset of thyroiditis

Neoplasms of the Thyroid

ADENOMAS CARCINOMAS

Papillary carcinoma (75% to 85% of cases)

  Follicular carcinoma (10% to 20% of cases)

Medullary carcinoma (5% of cases) Anaplastic carcinoma (<5% of cases

Parathyroid Glands

Hyperparathyroidism

PRIMARY HYPERPARATHYROIDISM Primary hyperparathyroidism is one of

the most common endocrine disorders, and it is an important cause of hypercalcemia

Adenoma: 75% to 80% Primary hyperplasia (diffuse or nodular):

10% to 15% Parathyroid carcinoma: less than 5%

Secondary Hyperparathyroidism Renal failure inadequate dietary intake of calcium,

steatorrhea, and vitamin D deficiency, may also cause this disorder

Hypoparathyroidism

Hypoparathyroidism results from decreased secretion of PTH or end-organ insensitivity (pseudohypoparathyroidism) due to congenital or acquired conditions. The disease is clinically characterized by hypocalcemia and hyperphosphatemia.

Hypoparathyroidism

There are many possible causes of deficient PTH secretion resulting in hypoparathyroidism: Surgically induced hypoparathyroidism Congenital absence of all glands Familial hypoparathyroidism Idiopathic hypoparathyroidism

Hypoparathyroidism

The major clinical manifestations of hypoparathyroidism are referable to hypocalcemia and are related to the severity and chronicity of the hypocalcemia The hallmark of hypocalcemia is tetany Mental status changes can include emotional

instability, anxiety and depression, confusional states, hallucinations, and frank psychosis.

Hypoparathyroidism

Intracranial manifestations include calcifications of the basal ganglia, parkinsonian-like movement disorders.

Ocular disease results in calcification of the lens leading to cataract formation.

Cardiovascular manifestations include a conduction defect