Transcript Vývojové poruchy dutiny ústní

The endocrine system
Pituitary gland
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Anterior lobe
Posterior lobe
Endocrine abnormalities
Local mass effect
Hyperpituarism
Caused by adenomas:
 Growth hormone
 Adrenocorticotropic hormone
 Prolactin
 Rare – thyroid stimulating hormone, gonadortropin
 25% adenomas – non-functional (causes
HYPOpituarism by compression)
 Microadenomas, macroadenomas (10mm)
 Nuclear atypia is NOT sign of malignancy
 Ultrastructurally – secretory granules
Somatotropic adenomas
 Acromegaly – in adults
 Gigantism – prior to closure of epiphyses
 Granulated, eosinophilic cells – eosinophilic adenoma
Prolactinomas
 Hypogonadism
 Galactorrhea
 Granulated acidophilic or chromophobic cells –
chromophobic adenoma
Corticotroph tumors
 Cushing’s syndrome
 Basophilic cells – basophilic adenoma
Hypopituarism
Caused by
1) hypothalamic lesions:
 Craniopharyngioma
 Glioma
 Germinoma
2) pituitary lesions:
 Nonsecretory adenomas
 Sheehan’s syndrome
 Empty sella syndrome
Clinically – variable
 Hypogonadotropism
 Hypothyroidism, etc.
Hypothalamic lesions –
craniopharyngioma
 Benign cystic tumor
 Calcifications
 Squamous epithelial cells and reticular stroma
Nonsecretory chromophobe
pituitary adenoma
 Mass effect (visual problems, headache)
 Chromophobic or oncocytic forms exists
Sheehan’s syndrome
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Associated with obstetric haemorrhage or shock
Caused by infarction of anterior pituitary
Gonadal failure – inability to lactate
ACTH, TSH deficiency
Healing of necrosis – fibrous tissue
Posterior pituitary syndrome
 Excess or deficiency of antidiuretic hormone – ADH
 Caused by suprasellar/hypothalamic lesions
Posterior pituitary syndrome
Excess of ADH
 Abnormal resorption of water, hyponatremia and inability
to excrete diluted urine
Caused by ectopic ADH secretion:
 Non-endocrine neoplasms (small cell carcinoma of the
lung)
 Non-neoplastic pulmonary diseases (TBC,
pneumonia)
 Primary CNS lesions (infarcts, meningitis,
haemorrhage)
Posterior pituitary syndrome
ADH deficiency (Diabetes insipidus)
Inability to concentrate urine:
 Polyuria
 Polydipsia
 Hypernatremia
Thyroid gland
 Hyperthyroidism
 Hypothyroidism
 Goitre – focal, diffuse
Hyperthyroidism (thyrotoxicosis)
 Increased levels of triodothyronine (T3), thyroxine (T4)
 Clinically: wide-eyed gaze, tachycardia, palpitations,
nervouseness, weight loss (increased appetite), moist
hand, tremor, peripheral vasodilatation
 Associated with diffuse hyperplasia (Graves’ disease) or
with toxic multinodular goitre or toxic adenoma
 May be associated with struma ovarii (teratoma)!!
Graves’ disease
 Autoimmune process
 Presence of thyroid stimulating antibody (TSAb) and
thyrotropin binding inhibitor immunoglobulin (TBII)
 Associated with other autoimmune diseases
 Presence of hyperplasia of foIlicular epithelium ,
depletion of colloid and lymphoid aggregates
Hypothyroidism
Cretinism (during infancy)
 Endemic form
 Sporadic form
 Physical an mental retardation
Myxoedema (in adults)
Slowing of physical and mental activity, fatigue and apathy
Signs - periorbital oedema, coarsening of skin,
cardiomegaly, accumulation of mucopolysaccharides in
dermis
Various causes - idiopathic primary, inflammation –
Hashimoto thyroiditis, etc.
Thyroiditis
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Hashimoto’s thyroiditis
De Quervain’s thyroiditis
Riedel’s fibrosing thyroiditis
Lymphocytic thyroiditis
Infectious thyroiditis
Hashimoto’s thyroiditis
 Autoimmune disorder
 Female predominance
 Defect in suppressor T cells, production of
autoantibodies
 Associated with other autoimmune disease (SLE,
Sjögren sy, rheumatoid arthritis…)
 Microscopically – dense lymphocytic infiltrate,
germinal centers, abundant eosinophilic oncocytes
(Hürtle cells)
De Quervain’s subacute
granulomatous thyroiditis
 Also known as giant cell thyroiditis
 Probably viral etiology
 Destruction of follicles, neutrophil infiltrate,
multinucleate giant cells
 Recovery in 6-8 weeks
Subacute lymphocytic thyroiditis
 Nonspecific lymphoid infiltration
 Without germinal centre
 In women in postpartum period
Riedel’s fibrosing thyroiditis
 Thyroid replaced by fibrous tissue
 Fibrous tissue extends and penetrate into the
surrounding neck structures
 May be mistaken for infiltrating neoplasm
Tumors
Benign – adenomas
 Well demarcated
 Fibrosis
 Haemorrhage
 Calcifications
 Hürtle cell adenoma - oncocytic
 Usually „cold“
Malignant - carcinomas
 See transparency
Parathyroid gland
Primary hyperparathyroidism
 Hypersecretion of parathormone
 Caused by adenoma (80%), hyperplasia (15%),
carcinoma (5%)
 Bone resorption, hypercalcemia – osteoporosis,
muscle weaknes, nephrolithiasis, ulcers, pancreatitis,
headache, depression
Secondary hyperparathyroidism
 In patients with renal failure
 Compensatory hypersecretion of parathormone (reaction
to phosphate retention and hypocalcemia)
Parathyroid gland - tumors
Adenoma
 Solitary, encapsulated – compression of adjacent gland
 No stromal fat
 Composed predominatly of chief cells
 Part of MEN I, MEN II
Carcinoma
 Rare
 Invasion, metastases
Hyperplasia
 All glands
 Fat cells interspersed
Hypoparathyroidism,
pseudohypoparathyroidism
Hypoparathyroidism
 Multiple etiology (surgical removal, autoimunne
destruction, congenital…)
 Tetany, neuromuscular excitability, paraesthesiae
psychosis
Pseudohypoparathyroidism
 Rare
 Abnormality PTH receptors, loss of responsiveness,
hypocalcemia
 Compensatory parathyroid hyperfunction
Adrenal cortex - hyperfunction
Three syndromes:
 Cushing’s syndrome
 Hyperaldosteronism
 Adrenogenital syndromes
Cushing’s syndrome
Causes:
 Administration of exogenous glucocorticoids – most
common
 Pituitary hypersecretion of ACTH (Cushing’s disease) –
adenoma
 Ectopic ACTH secretion – small cell carcinoma !!
Histology:
 Crooke’s hyaline changes within pituitary basophils
Clinically:
 Central obesity, moon facies, fatigability, hirsutism,
hypertension, osteoporosis, cutaneous striae
Hyperaldosteronism
Conn’s syndrome:
 Adenoma/hyperplasia
 Excessive production of aldosterone – low plasma renin,
sodium retention, hypertension, loss of potassium,
muscular weakness, cardiac arrhytmias, metabolic
alkalosis, tetany
Secondary:
 Reduced glomerular perfusion (fail in blood volume) –
activation of renin angiotensin system – stimulation of
aldosterone secretion
 Most common
Adrenogenital syndromes
 Variable manifestation (virilization, pubertax praecox,
hermaphroditism, pseudohermaphroditism)
 Autosomal recessive trait
 Most often – deficiency of 21-hydroxylase - virilization
Hypofunction of adrenal cortex
 Adrenal crisis
 Addison’s disease
 Secondary insufficiency
Primary acute adrenocortical
insufficiency
 Rapid withdrawal of steroids
 Massive destruction of steroids – WaterhouseFriderichsen syndrome:
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During septic meningococcal infection
Massive hemorrhage
Hypotension
Shock
DIC
Addison’s disease (chronic
adrenocortical insufficiency)
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Autoimunne
Infection (TBC, fungi, etc.)
Metastatic cancer (lung, stomach, etc.)
Clinically:
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Anorexia
Weakness
Cutaneous hyperpigmentation
ACTH elevation (in primary insufficiency)
Secondary insufficiency
 decreased production of ACTH, absence of
hyperpigmentation, normal aldosterone levels
Adrenal medulla
Phaeochromocytoma
 Catecholamine production – hypertension
 85% in medulla (extra-adrenal tumors designated
paragangliomas)
 Sporadic (90%) or associated with familial syndromes
(MEN, von Hippel-Lindau, von Recklinghausen)
 Histologically – pleomorphism, mitotic activity - however
there are no reliable histological predictors of
malignancy!!
 Only criterion of malignancy – metastasis
Other tumors – neuroblastoma, ganglioneuroma
Multiple Endocrine Neoplasia
MEN
MEN I (Wermer’s syndrome)
 Parathyroid (hyperplasia, adenoma)
 Pancreas (islet cell tumors)
 Pituitary (adenoma)
MEN II (Sipple’s syndrome)
 Medullary thyroid carcinoma
 Phaeochromocytoma
 Parathyroid adenoma/hyperplasia
MEN III
 MEN II and neuroma/ganglioneuroma
All MENs – autosomal dominant trait