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EMBRYOLOGY There are two distinct functional units in the adrenal gland: cortex and medulla. The cortex is derived from the mesoderm. At about the fifth week of life the fetal cortex develops; this is subsequently surrounded by a second wave of mesothelial ce...

Extra-adrenal paraganglia Paraganglia are neuroendocrine cells associated with the autonomic nervous system. They can be adrenal (medulla) or extra-adrenal. Extra-adrenal paraganglia occur throughout the sympathetic nervous system and along some branches of t...

Ganglioneuroma Defi nition Ganglioneuromas (GNs) are benign di ff erentiated tumours of neural crest-derived cells in the autonomic nerves. Incidence GNs are rare, a ff ecting one per million of the population. Most are sporadic but they can be associated with ...

INCIDENTALOMA Definition An asymptomatic adrenal mass detected on imaging not performed for suspected adrenal disease is termed an inciden taloma. The aetiology includes benign and malignant tumours of the cortex and medulla or of extra-adrenal origin. These ...

INTRODUCTION Two major systems are held responsible for the regulation of homeostasis of the human body: the endocrine and neuroen docrine systems. Both interact with their target organs or target tissues via secretion of ubiquitous messenger molecules, whic...

Learning objectives Adrenal gland To understand the investigation and diagnosis of disorders • of the adrenal gland To know of the principles and postoperative management • of adrenalectomy Pancreatic endocrine disorders and gastrointestinal neuro endocrine tu...

MUL TIPLE ENDOCRINE NEOPLASIA MEN is a group of heterogeneous disorders characterised by a predisposition of benign and malignant tumours involving two - or more endocrine glands. There are four varieties, the most common being MEN /uni00A0 1 and MEN /uni00A...

Metastases Definition Adrenal metastases are not uncommon and often portend - disseminated incurable disease. Primary tumours that commonly spread to the adrenals include lung, renal, gastric, breast and colorectal cancers. The decision regarding surgical inter...

Minimally invasive adrenalectomy Transperitoneal laparoscopic adrenalectomy Familiarity with the anatomy of the adrenal region is essential and it should be noted that the approaches for the right and left sides are distinct. Careful haemostasis is essential ...

Multiple endocrine neoplasia type 1 Epidemiology and genetics MEN /uni00A0 1 is an autosomal dominant inherited disorder. It was originally called Wermer’s syndrome. It is rare (1 in 30 /uni00A0 000) in the general population but accounts for 10% of primary h...

Multiple endocrine neoplasia type 2A . ). In Epidemiology and genetics MEN /uni00A0 2A has a prevalence of 1 in 25 /uni00A0 000 people. It is an auto - somal dominant condition caused by germline mutations in RET , which encodes a transmembrane tyrosine kinas...

Multiple endocrine neoplasia type 2B Epidemiology and genetics - This has a prevalence of 0.2 in 100 /uni00A0 000. MEN /uni00A0 2B is also caused by RET mutations, half of which are de novo . Clinical presentation A variant of MEN /uni00A0 2 characterised ...

Multiple endocrine neoplasia type 4 Described in 2006, this is an exceptionally rare autosomal dominant syndrome consisting of MEN1 -associated tumours (parathyroid and pituitary) in association with tumours of the kidney , adrenal glands and reproductive orga...

Neuroblastoma Definition Neuroblastoma (NB) is the most common and deadliest solid extracranial malignancy in children. It is derived from the primitive nerve cells (neuroblasts) of the sympathetic nervous system, derived from the neural crest. It is often ter...

Non-functioning pancreatic neuroendocrine tumours Definition P-NETs are clinically classified as non-functioning (NF-P NET) when they do not cause a clinical syndrome. Incidence NF-P-NETs account for 60–90% of all P-NETS. Pathology NF-P-NETs cannot be distingui...

OF THE STOMACH AND SMALL INTESTINE Embryology and physiology The embryology of the intestine is developed from the foregut, midgut and hindgut. The bronchus and lungs are also developed from the foregut. Throughout these tissues are APUD neuroendocrine cells....

PANCREATIC NEUROENDOCRINE TUMOURS Introduction As with neuroendocrine cells in the intestine, pancreatic neuro endocrine cells are believed to be derived from pluripotent stem cells that di ff erentiate into endocrine cells. From these, tumours may arise tha t ...

PHYSIOLOGY The outer zona glomerulosa secretes the C21 steroid aldosterone. The zona fasciculata secretes (C21 steroid) cortisol and the inner zona reticularis secretes (C19 steroid) androgens. Aldosterone regulates sodium–potassium homeostasis; it promotes so...