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Pathology of the Endocrine System

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Pathology of the Endocrine System M3422-Ch10.qxd 23/4/07 10:57 AM Page 205 Pathology of the endocrine 10 system Objectives In this chapter, you will learn to: • Describe disorders of the pituitary gland leading to hyperpituitarism and hypopituitarism. • Describe the causes and patterns of thyroid dysfunction. • Describe the causes and patterns of parathyroid dysfunction. • Understand Cushing’s syndrome and other disorders of adrenal hyperfunction. • Understand the importance of adrenal hypofunction and crisis. • Recall the pathology and complications associated with diabetes mellitus. • Briefly describe endocrine tumours including the important multiple endocrine neoplasia syndromes. DISORDERS OF THE PITUITARY • Neurons of the supra-optic nucleus (projecting into the posterior pituitary), which release The pituitary (hypophysis) is a small (500–1000 mg), ADH. bean-shaped gland lying in the sella turcica in the • Chromaffin cells of the adrenal medulla, which base of the skull. It is composed of two parts: release epinephrine (adrenaline). 1. Anterior lobe (adenohypophysis)— synthesises and secretes a number of The anterior pituitary: hormones (Fig. 10.1), most of which act on hyperpituitarism other endocrine glands. Hyperpituitarism is defined as excessive secretion of 2. Posterior lobe (neurohypophysis)—stores and one or more of the pituitary hormones. Its most secretes two hormones synthesised in the common causes are functioning (hormone-secreting) hypothalamus: antidiuretic hormone (ADH; adenomas of the anterior lobe. vasopressin) and oxytocin. This lobe is in direct continuity with the hypothalamus, to which it is Anterior lobe adenomas connected via the pituitary stalk. Anterior lobe adenomas comprise about 10% of all intracranial tumours (posterior lobe adenomas do Secretion of the pituitary hormones is regulated by not occur). These tumours do not usually metastasise, neural and chemical stimuli from the hypothalamus, but they are often life threatening because of their diseases of which cause secondary abnormalities in position and ability to secrete excess hormone. pituitary function. This cooperation between the nervous system and Effects of pituitary adenomas endocrine apparatus is referred to as neuroendocrine Pituitary adenomas cause problems because of a com- signalling. Figure 10.2 shows the integration of signals bination of endocrine effects (excessive secretion of a between the hypothalamus, pituitary and thyroid particular hormone) and compressive effects, caused gland in the release of thyroid hormones, with feed- by an increase in local pressure of the following: back loops acting at each level. Neuroendocrine cells are defined as those that • Remainder of the pituitary → hypopituitarism. release a hormone in response to a neural stimulus. • Optic chiasm → visual field defects, notably Important examples include: bitemporal haemianopia. 205 M3422-Ch10.qxd 23/4/07 10:57 AM Page 206 Pathology of the endocrine system • Brain (large tumours) → distortion of the cavity of third midbrain with raised intracranial pressure and ventricle hydrocephalus. optic mammillary • Dura → headaches. chiasma body • Cavernous sinus → CN III, IV or VI nerve dura hypothalamus antidiuretic palsies. mater hormone (ADH) oxytocin The endocrine effects depend on which hormone is being excessively secreted (see below). Investigations: anterior posterior • Imaging—plain X-ray (can detect enlargement pituitary pituitary of sella turcica and erosion of the clinoid processes) and MRI (for visualisation and sizing of the tumour; this is superior to CT scanning). sphenoid • Hormone assays (e.g. growth hormone, bone growth hormone (GH) sella prolactin prolactin). turcica follicle stimulating hormone (FSH) • Functional testing of the pituitary–adrenal axis, luteinizing hormone (LH) β endorphin e.g. ACTH stimulation test in which a dose adrenocorticotrophic hormone (ACTH) of adrenocorticotrophic hormone (ACTH) is thyroid stimulating hormone (TSH) given and the plasma cortisol response melanocyte stimulating hormone (MSH) measured. • Visual field assessment. Fig. 10.1 Pituitary and hypothalamus, with the hormones released. Types of functioning adenomas input from Functioning adenomas may produce any of the ante- higher centres rior lobe (adenophyseal) hormones but the majority +or− indirect produce prolactin (prolactinomas–lactotroph ade- feedback +or− +or− loop nomas), growth hormone (somatotroph adenomas) hypothalamus or ACTH (corticotroph adenomas). Prolactinomas Abnormally raised serum prolactin levels are associ- releasing hormone ated with menstrual irregularity and infertility in e.g. TRH women, and with ejaculatory failure or impotence in +or− men. Mild prolactin increases are seen with compres- +or− sion of the hypothalamus by any pituitary adenoma pituitary (the ‘stalk effect’). Galactorrhoea is present in about 30% of affect- ed women, but it is rare in men because oestrogen trophic hormone direct priming is required for lactation. e.g. TSH feedback short loop Somatotroph adenoma feedback + loop This results in hypersecretion of growth hormone, the target gland target gland hormone effects of which depend on the developmental stage e.g. thyroid e.g. thyroxine of the affected individual: • Pre-epiphyseal union (prepubertal) leads to Fig. 10.2 Schematic representation of the integration between the higher centre, hypothalamus, pituitary and target organ signalling. The gigantism (giantism), i.e. excessive growth in a example is for thyroid function, highlighting the feedback loops that regular and initially well-proportioned manner. control hormone release at each level. (TRH, thyrotrophin releasing Most giants also show some features of hormone, TSH, thyroid stimulating hormone). (Adapted with permission from Essential Endocrinology, 4th edn, by Brook and acromegaly with disproportionate enlargement, Marshall, Blackwell Publishing, 2001). e.g. of the hands and jaw. 206 M3422-Ch10.qxd 23/4/07 10:57 AM Page 207 Disorders of the pituitary 10 • Postepiphyseal union (adults) leads to resulting in the excessive secretion of glucocorticoids acromegaly, which is characterised by causing Cushing’s syndrome, the effects of which are enlargement of the hands, feet, and head. They described later (see page 219). may also present with secondary diabetes (growth hormone is an insulin antagonist) or Other functioning adenomas cardiovascular effects (Fig. 10.3). Other endocrine secreting adenomas, e.g. of thyroid- stimulating hormone (TSH), luteinizing hormone There are three types of treatment: (LH) and follicle-stimulating hormone (FSH), are • Surgery—hypophysectomy (transfrontal or extremely rare. transphenoidal), especially where there are signs of compression of adjacent structures. This The anterior pituitary: usually only debulks the tumour, with further hypopituitarism (usually drug) therapy required. Hypopituitarism is defined as insufficient secretion of • Radiotherapy—fewer complications than surgery the pituitary hormones. The clinical features depend but less successful. on the patient’s age and on the type and severity of the • Drug therapy—bromocriptine (dopamine hormone deficiencies (Fig. 10.4). agonist) and octreotide (somatostatin analogue) Hypopituitarism can be caused by either hypo- can lower growth hormone levels in thalamic lesions or pituitary lesions. uncomplicated acromegaly. Hypothalamic lesions are: Corticotrophin adenoma • Idiopathic deficiency of one or more of the Overproduction of ACTH by the pituitary gland releasing factors, e.g. gonadotrophin-releasing (Cushing’s disease) causes adrenal hyperplasia, hormone (GnRH; Kallmann’s syndrome), growth-hormone releasing factor (GHRH) or, more rarely, thyrotrophin-releasing hormone skull brain (TRH) or corticotrophin-releasing factor (CRF). - enlarged head - mental circumference disturbances • Infarction. - insomnia • Inflammation, e.g. sarcoidosis, tuberculous face - large lower jaw meningitis. - spaces between lower • Suprasellar tumours, e.g. craniopharyngioma or, heart teeth due to jaw growth more rarely, pinealoma, teratoma or a secondary - large nose - enlarged - large tongue tumour from another site. Pituitary lesions are: liver and kidneys - enlarged organs • Idiopathic deficiency of one or more of the pituitary hormones. • Non-functioning chromophobe pituitary adenomas—adenomas of the anterior pituitary hands (usually derived from non-hormone-secreting - large, square and spade like blood pressure chromophobe cells), which may cause - hypertension hypopituitarism by compression or obliteration blood bones of normal pituitary tissue. - hypercalcaemia - predisposes to • Sheehan’s syndrome—ischaemic necrosis of osteoarthritis the anterior pituitary due to hypotensive shock occurring as a result of obstetric skin - increased greasy haemorrhage. sweating • Empty sella syndrome—an enlarged, empty sella - temperature feet intolerance turcica that is not filled with pituitary tissue. This - large and wide may be a primary anatomical variant or it may follow spontaneous infarction, surgery, or Fig. 10.3 Features of acromegaly. radiotherapy of a tumour. 207 M3422-Ch10.qxd 23/4/07 10:57 AM Page 208 Pathology of the endocrine system Fig. 10.4 Clinical features associated with specific forms of hypopituitarism. Fig. 10.4 Clinical features associated with specific forms of hypopituitarism Hormone Tests to exclude hypofunction deficiency Clinical features of anterior pituitary Gonadotrophin Prepubertal: LH reserves adequate if: deficiency • Failure to enter puberty • Males have a normal • Undescended testes
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