53 - Diseases of the endocrine system Flashcards by David Thomas

Pituitary gland - features

Situated in Sella Turcica beneath hypothalamus

Ant part (75%) - outpouching of oral cavity

Post part (25%) down growth of hypothalamus

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Ant. pit. hypofunction

Tumours - non-sec adenoma, metastatic carcinoma
Trauma
Infarction
Inflammation - granulomatous, autoimmune, other infections
Iatrogenic

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1° pituitary tumours

Most are adenomas + benign
Derived from any hormone producing cell
Functional clinical effect 2° to hormone being produced
Local effects due to pressure on optic chiasma or adjacent pituitary

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Types of pit adenoma

Prolactinoma (most common) - galactorrhoea and menstrual disturbance

Growth hormone secretion - gigantism in children. acromegaly in adults

ACTH secreting - Cushing’s syndrome

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Thyroid anatomy

Bilobed joined by isthmus
Thin fibrous capsule
C5,6,7

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Thyroid embryology

Main part migrates from foregut to ant. neck (remnant is foramen caecum at junction 2/3 and post 1/3 of tongue

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Ectopia and heterotopia

Lingual thyroid - most common at base of tongue
>75% of patients with this are asymptomatic.

The others have hypothyroid and 10% of these cretinism

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Thyroglossal duct cyst

Persistent track representing the embryological migratory path of thyroid anlage of the ant. neck

7% of adults
Asymptomatic midline neck mass

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Acute thyroiditis

Acute inflammation of the thyroid parenchyma associated with local/systemic infection

Generalised sepsis
Fever, chills, malaise, pain, swelling of ant. neck

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Palpation thyroiditis

Microscopic granulomatous foci centred on thyroid follicles

2° to rupture of thyroid follicles due to palpation or surgery

Patients almost always have a thyroid nodule

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Riedel thyroiditis

Rare fibrosing form of chronic thyroiditis

Fibrosing disorder may also affect retroperitoneum, lung, mediastinum, biliary tree, pancreas, kidney, subcutis.

Present with firm goitre

Symptoms inc. dysphagia, hoarseness, stridor.

Mistaken for malignant neoplasm

Benign self-limited disease

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Chronic lymphocytic thyroiditis (Hashimoto’s) - what is it?

Autoimmune chronic inflammatory disorder associated with diffuse enlargement and thyroid autoantibodies

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Chronic lymphocytic thyroiditis (Hashimoto’s) - risk factors

Female>male
Peak age = 59
Diffusely enlarged non-tender gland
Serum thyroid antibodies elevated
Lymphocytic infiltration of thyroid parenchyma, often with germinal centre formation
Causes hypothyroid
80-fold risk of thyroid lymphoma
Increased risk of papillary carcinoma of the thyroid

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Diffuse hyperplasia (Grave’s) - what is it?

Autoimmune process results in clinical hypothyroidism and diffuse hyperplasia of the follicular epithelium

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Diffuse hyperplasia (Grave’s) - epidemiology

1% worldwide
Female>male
Peak in third and fourth decades
80% of hypothyroidism cases
Symptoms of hyperthyroidism
Thyroid diffusely enlarged
T3 and T4 elevated w/ TSH suppressed
Thyroid autoantibodies especially TSH Ig
May develop permanent hypothyroidism

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Diffuse hyperplasia (Grave’s) - clinical presentation

Pretibial myxoedema, hair loss, wide-eyed stare or proptosis, tachycardia, hyperactive reflexes

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Multinodular goitre

Enlargement of thyroid with varying degrees of nodularity
Most patients are euthyroid
Dominant nodule may be mistaken clinically for thyroid carcinoma
Tracheal compression or dysphagia may develop with large nodules

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Follicular adenoma

Benign encapsulated tumour with evidence of follicular cell differentiation

F>M
Wide age range - 50,60
Painless neck mass for years
Solitary nodule involved one lobe
Usually cold nodule on radioactive iodine imaging

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Papillary carcinoma - who? prevalence?

commonest type of tyroid carcinoma

F>M
43 yo mean

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Papillary carcinoma - risk factors

Familial autosomal dominant non-medullary thyroid carcinoma
FAP
Cowden's syndrome
Therapeutic radiation
Radiation exposure

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Papillary carcinoma - pathogenesis

Activation of RET or NTRK1

Variety of chromosomal translocations or inversions

BRAF V600E
RAS mutations

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Papillary carcinoma - macroscopic appearance

Ill defined
Infiltrative
Some encapsulated
May be cystic
Granular

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Follicular neoplasms - types

Follicular adenoma
Minimally invasive follicular carcinoma
Widely invasive follicular carcinoma
Hurthle cell neoplasm

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Follicular carcinoma - %%%

10-20% of all thyroid cancers
90% present with solitary nodule in thyroid

Minimally invasive = 5% mets
Widely invasive = 60% mets

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Follicular neoplasms - minimal vs widely invasive

min: completely encapsulated, invasion only detectable histologically wide: macroscopic evidence of invasion. Widespread invasion histologically. Minimally invasive but tumour invades >4 capsular blood vessels.

Hurthle cell carcinoma - what is it?

Recognised large acidophilic cells in canine thyroid Parafollicular or C cells

Hurthle cell carcinoma - who?

3% of all differentiated thyroid carcinomas Med age: 53 (24 -85yo tho) Sex F:M, 7:3

Hurthle cell carcinoma - clinical behaviour

Significant incidence of lymph node mets Common haemato spread to bone, liver and lung

Insular carcinoma - who?

Elderly patients of mean age 58

Insular carcinoma - mets?

Lymph node and distant mets in up to 50% up to 60% will die

Anaplastic carcinoma - who?

Older patients | Mean age 65

Anaplastic carcinoma - clinical presentation

Longstanding history of goitre | Usually inoperable

Anaplastic carcinoma - prognosis

Median survival is months

Medullary carcinoma - what is it?

Malignant tumour showing differentiation to parafollicular C cells

Medullary carcinoma - genetic basis

``` 70-80% are sporadic 20-30% are AD MEN2a and B Familial MTC Mutations in RET gene ```

Primary thyroid lymphoma - what is it?

Primary lymphoma arising within thyroid gland often associated with lymphocytic thyroiditis

Primary thyroid lymphoma - who?

2% of thyroid neoplasms | Mean age 70

Primary thyroid lymphoma - clinical presentation

Mass in thyroid Rapid enlargement Pain Dysphagia Associated cervical lymphadenopathy

Primary thyroid lymphoma - prognosis

Dependent on histology + stage 60% survival

Where do thyroid tumours met to?

``` Renal cell carcinoma Melanoma Small cell lung cancer Neuroendocrine carcinoma Breast cancer ```

Primary hyperparathyroidism definition

Excessive section of PTH from one of more glands

Secondary hyperparathyroidism

Hyperplasia of glands with elevated PTH in response to hypocalcaemia Renal insufficiency, malabsorption, vit D deficiency

Tertiary hyperparathyroidism

Adenoma in association with longstanding secondary hyperparathyroidism

Primary hyperparathyroidism - pathogenesis

Ageing Ionising radiation MEN 2a

Primary hyperparathyroidism - types

Single adenoma (85%) Diffuse chief or clear cell hyperplasia (10%) Carcinoma (5%)

Primary chief cell hyperplasia - definition

Non-neoplastic increase in parathyroid parenchymal cell mass within all parathyroid tissue w/o a known stimulus

Primary chief cell hyperplasia - who?

17 per 1,000,000 | Peak age = 50-70 yo

Primary chief cell hyperplasia - symptoms

Fatigue, lethargy, anorexia, weakness, vomiting, bones, stones and ab moans Elevated calcium, low phosphorous high parahormone

Primary chief cell hyperplasia - treatment

surgery

Parathyroid adenoma - what is it?

Encapsulated benign neoplasm of parathyroid cells

Parathyroid adenoma - who?

1 per 1,000

Parathyroid adenoma - symptoms

Hypercalcaemia

Parathyroid adenoma - risk factors

MEN1 and MEN2 and hyperparathyroidism and jaw tumour syndrome

Parathyroid adenoma - clinical presentation

Single enlarged parathyroid gland, remaining gland suppressed and small

Parathyroid carcinoma - what is it?

Malignant tumour derived from parathyroid parenchymal cells

Parathyroid carcinoma - prevalence

5% of primary hyperparathyroids

Parathyroid carcinoma - symptoms

Hypercalcaemia

Parathyroid carcinoma - prognosis

50% 10 year survival rate

Parathyroid carcinoma - treatment

Surgery

Adrenal congenital hypoplasia - what is it?

Reduced volume of adrenocortical tissue leading to adrenal cortical insufficiency

Adrenal congenital hypoplasia - prevalence?

Rare

Adrenal congenital hypoplasia - risk factors

Family

Adrenal congenital hypoplasia - prognosis

poor if untreated

Adrenal congenital hypoplasia - who?

Male predominance often XL

Adrenal congenital hypoplasia - clinical presentation

Hypoadrenalism

Adrenal congenital hypoplasia - used to be fatal now treatment is...

Glucocorticoid and mineralocorticoid replacement

Congenital adrenal hyperplasia - what is it?

Inherited disorder caused by deficiency of enzymes req. for synthesis of glucocorticoids and mineralocorticoids

Congenital adrenal hyperplasia - pathogenesis

21 hydroxylase deficiency accounts for more than 90%

Congenital adrenal hyperplasia - clinical presentation

Deficiencies of cortical and aldosterone secretion Genital abnormality in females, nomral in males Virilisation and hyperandrogenism Advanced bone growth with premature epiphyseal maturation leading to short adult stature

Addison's disease - what is it?

Primary adrenal cortical insufficiency caused by adrenal dysgenesis, adrenal destruction

Addison's disease - who?

5.3 per million pop | High mortality if not diagnosed

Addison's disease - pathogenesis

Autoimmune form most common | TB more in developing world

Addison's disease - clinical presentation

hyperpigmentation, postural hypotension and hyponatraemia

Addison's disease - treatment

Long term steroid replacement

Adrenal cortical nodule - what is it?

Benign non-functional nodules of adrenal cortex

Adrenal cortical nodule - who?

1.5 and 3% of population Elderly, hypotensive and diabetic

Adrenal cortical nodule - how often diagnosed?

Incidental on radiograph

Adrenal cortical nodule - treatment

none required

Adrenal cortical adenoma - what is it?

Benign neoplastic proliferation of adrenal cortical tissue

Adrenal cortical adenoma - incidence

1-5% of pop

Adrenal cortical adenoma - symptoms related to endocrine hyperfunction

Hypertension Cushing's Virilisation

Adrenal cortical adenoma - what do they release sometimes? what does this cause?

Aldosterone-producing tumours can cause Conn's syndrome Cortisol-producing reduce Cushing's syndrome Rare tumours cause virilisation

Adrenal cortical adenoma - look like

Unilateral solitary masses Average size 2cm Well-circumscribed yellow/brown nodules

Adrenal cortical carcinoma - what is it?

Malignant counterpart of adrenal adenoma

Adrenal cortical carcinoma - who?

3% of endocrine neoplasms | 1 per million pop

Adrenal cortical carcinoma - symptoms

Hormone excess | Ab mass

Adrenal cortical carcinoma - prognosis

age, stage | 5yr = 70%

Phaeochromocytoma - what is it?

Catecholamine-secreting tumour arising from adrenal medulla

Phaeochromocytoma - who?

8 per million

Phaeochromocytoma - pathogenesis

Most sporadic but familial = MEN2a and 2b von recklinghausen's disease and von hippel-lindau disease symptoms of hypertensions, palpitations, headaches and anxiety.

Phaeochromocytoma - tests

elevated urine catecholamines, adrenaline, noradrenaline

Phaeochromocytoma - prognosis

excellent when benign and surgically managed

53 - Diseases of the endocrine system Flashcards

(92 cards)