Thursday, September 25, 2008

Craniopharyngioma
9% of all paediatric cranial tumours
due to persistence of Rathke's pouch

- slow growing benign suprasellar tumour
- arise from cells along pituitary stalk
- the nest of epithelium cells at the rathke's pouch are embryonic precursors to anterior pituitary gland
- craniopharyngiomas are cystic or partiallycystic

Symptoms:
1. precocious puberty
2. headache
3. growth failure
4. bitemporal hemianopia

McCune Albright Syndrome:
Most common cause of GnRH independent precocious puberty
Predominantly occurs in females, may occur in males
Suspected if 2/3 of the triad of:
1. polyostotic fibrous dysplasia (especially lower limbs, 50% facial involvement)
2. endocrine abnormality e.g. precocious puberty, autonomous hyperfunction of other endocrine glands
3. cafe au lait spots

pathophysiology:
mutation resultin ig in Gsalpha subunit activating mutation

Presentation:
1. asymmetrical ovarian cysts
2. suppressed LH, FSH (due to autonomous hyperfunction of the endocrine glands)
Other endocrine abnormalities: think pituitary
1. hyperthyroid (TSH)
2. cushing's syndrome (ACTH)
3. acromegaly (GH)
4. Hyperprolactinaemia (posterior pituitary)
5. primary hyperparathyoid (low phosphates due to decreased phosphate reabsorption at kidneys)
--> hypophosphotaemic rickets
--> repeated fractures and deformities (2* to fibrous dysplasia)
6. Cranial nerve compression (why?)

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