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SYB #2

SYB #2. Karl Clebak 2 April 2008. Case Presentation. 16 yo male with history of short stature, o ver the past year has been drinking and urinating more. In the past month increasing frequency of headaches, mainly in the right temporal area and noticing bitemporal visual field losses. Case.

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SYB #2

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  1. SYB #2 Karl Clebak 2 April 2008

  2. Case Presentation 16 yo male with history of short stature, o ver the past year has been drinking and urinating more. In the past month increasing frequency of headaches, mainly in the right temporal area and noticing bitemporal visual field losses.

  3. Case dADC MAP T2

  4. Case CT T1 weighted

  5. Differential Dx • Congenital anomalies • Arachnoid cyst and Rathke's cleft cyst. • Other Tumours • Pituitary tumour, Craniopharyngioma. metastasis, meningioma, epidermoid and dermoidtumour, hypothalamic-optic pathway glioma, hypothalamic hamartoma, teratoma. • Infectious/Inflammatory processes • Eosinophilicgranuloma, lymphocytic hypophysitis, sarcoidosis, syphilis and tuberculosis. • Vascular malformations • Aneurysm of the internal carotid or anterior communicating artery, arterio-venous malformation.

  6. Craniopharyngioma • Epidemiology • 0.13 to 2 new cases per 100,000 population per year • 1 to 3 total cases per 100,000 population • no variance by gender or race • bimodal age distribution with the peak incidence • in children at 5–14 years • adults at 65–74 years of age

  7. Craniopharyngioma • Clinical • Slow growing tumors • delay of 1–2 years between symptom onset and diagnosis • Symptoms at diagnosis • Raised intracranial pressure • headaches, nausea and vomiting either from mass effect or secondary hydrocephalus • Endocrine dysfunction • normally suppressed endocrine function, for example • hypothyroidism, orthostatic hypotension, short stature, diabetes insipidus, impotence and amenorrhoea, but there can be an exaggeration of endocrine function, precocious puberty in children and obesity in adults.

  8. Craniopharyngioma • Visual disturbance • classically a bitemporal hemianopia from inferior chiasmatic compression but alternatively patients may have a homonymous hemianopia, scotoma and optic atrophy with papilloedema. • Other presenting symptoms include chemical meningitis (from rupture of cyst contents into the subarachnoid space), seizures, poor school performance in children or emotional lability and apathy in adults.

  9. Etiology • 2 types • Adamantinomatoustumours • Children • resemble enamel forming neoplasm's in the oropharynx • Squamous papillary form • Predominantly seen in adults

  10. Theories of origin • The embryogenetic theory • adamantinomatous type arises from epithelial remnants of the craniopharyngeal duct or Rathke's pouch. The duct and pouch were derived from the stomadeum, which forms teeth primordia. • The metaplastic theory • this suggests that the squamous papillary type occurs as a result of metaplasia of squamous epithelial cell rests that are remnants of the part of the stomadeum that contributed to the buccal mucosa.

  11. Diagnostic Methods • Imaging • The classical appearance of a craniopharyngioma • sellar/para sellar part solid, part cystic calcified mass lesion • These tumours occur in the supra sellar (75%), supra and infra sellar (20%) and infra sellar (5%) regions • The calcification is best delineated on computerised tomography (CT) • Magnetic resonance imaging (MRI) with and without contrast will, however, more accurately delineate the extent of the tumour and, in particular, its involvement with the hypothalamus • It is the investigation of choice to plan the surgical approach. • Magnetic resonance angiography (MRA) is useful to not only delineate the course of the vessels, which can be through the tumour, but also to help differentiate a tumour from a possible vascular malformation

  12. Diagnostic • Endocrine • LH, TSH, cortisol • Ophthalmology assessment • Visual field assessment, visual acuity, visualize optic discs • Histology • Epithelial appearance • Hyalinised calcified structures, collagen, fibroblasts, foreign body giant cells and occasionally cholesterol clefts

  13. Treatment • Gross total resection • High morbidity when invade hypothalamus • Limited surgery aimed at debulking followed by radiotherapy • Remaining tumor on MRI treated with external beam radiotherapy • Can use stereotactic radiosurgery (gamma knife)

  14. Prognosis • Overall 5 year survival • 80% • Better in children • 85% 5 year survival

  15. References • Craniopharyngioma. Dynamed. Updated Updated 2008 Mar 18 12:26 PM. Accessed 31 March 2008. • Garnett, M et al. “Craniopharyngioma.” Orphanet Journal of Rare Diseases. 2007 Apr 10;2:18 • Pusey et al. MR of craniopharyngiomas: tumor delineation and characterization. Am. J. Roentgenol. August 1987. 149 (2): 383.

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