Neuroradiology craniopharyngioma

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Neuro radiological findings in Caraniopharyngioma

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Neuroradiology craniopharyngioma

  1. 1. Neuro Radiology - Craniopharyngioma Dr.Roopchand.PS Senior Resident Neurology
  2. 2. Introduction: • Benign (WHO grade I) neoplasms which typically arise in the sellar / suprasellar region. • Account for ~ 1 - 5% of primary brain tumours. • Can occur anywhere from floor of the third ventricle, to the pituitary gland. • Two pathological types and they differ in appearance, epidemiology and prognosis. o adamantinomatous (paediatric) o papillary (adult) o mixed: ~ 15%, but share imaging and prognosis similar to adamantinomatous
  3. 3. Epidemiology: • Bimodal distribution: • First peak between the ages of 10 - 14 years o Adamantinomatous type. • Second peak in young to middle-aged adults o Papillary type • Similar incidence in males and females.
  4. 4. Clinical presentation: • Headaches and raised ICP • Visual symptoms o 20% of children o 80% adults • Hormonal imbalances o short stature and delayed puberty in children o decreased libido o amenorrhoea o diabetes insipidus • Behavioural change due to frontal or temporal extension.
  5. 5. Pathology: • Arises from the Rathke’s cleft. • This histological appearances of the two subtypes are different. • Adamantinomatous: o In children o Reticular epithelial cells which have appearances reminiscent of the enamel pulp of developing teeth. o single or multiple cysts filled with thick oily fluid high in protein, blood products, and/or cholesterol, creating the so called "machinery oil". o "Wet keratin nodules" are a characteristic histological feature. o Calcification is usually present : ~ 90%
  6. 6. • Papillary: o Seen almost exclusively in adults o Formed of masses of metaplastic squamous cells . o "Wet keratin" is absent. o Cysts do form, but these are less of a feature, and the tumour is more solid. o Calcification is uncommon or even rare
  7. 7. Radiographic features: • Significant suprasellar component (95%), • involving both the suprasellar and intrasellar spaces (75%). • Purely suprasellar (20%), • Purely intrasellar location is quite uncommon (<5%). • Larger tumours can extend in all directions, frequently distorting the optic chiasm, or compressing the midbrain with resulting obstructive hydrocephalus. • Occasionallycan appear as intraventricular, homogeneous, soft-tissue masses without calcification (papillary sub type). The third ventricle is a particularly common location. • Rare / ectopic locations include: nasopharynx, posterior fossa, extension down the cervical spine.
  8. 8. Adamantinomatous: • Lobulated contour as a result of usually multiple cystic lesions. • Solid components are present. o Form a relatively minor component of the mass, • Enhance vividly on both CT and MRI. • Calcification is very common, but this is only true of the adamantinomatous subtype (90% are calcified) • Predilection to be large, extending superiorly into the third ventricle, and encasing vessels, and even being adherent to adjacent structures.
  9. 9. • CT • cysts o typically large and a dominant feature o near CSF density • solid component o soft tissue density o vivid enhancement • calcification o seen in 90% o typically stippled and often peripheral in location
  10. 10. • MRI • cysts: variable but ~80% are mostly or partly T2 hyperintense • solid component o T1: iso to lightly hypointense to brain o T1 C+: vivid enhancement o T2: variable / mixed • calcification o difficult to appreciate on conventional imaging o susceptible sequences may better demonstrate calcification • MR angiography: may demonstrate displacement of the A1 segment of the anterior cerebral artery • MR spectroscopy: cyst contents may show a broad lipid spectrum, with an otherwise flat baseline 6
  11. 11. T1 T1C
  12. 12. Papillary : • Papillary craniopharyngiomas tend to be more spherical in outline and usually lack the prominent cystic component. • Most are either solid or contain a few smaller cysts. • Calcification is uncommon or even rare in the papillary subtype
  13. 13. • CT • cysts o small and not a major feature o near CSF density • solid component o soft tissue density o vivid enhancement • calcification o uncommon - rare
  14. 14. MRI: • cysts o when present they are variable in signal o 85% T1 hypointense • solid component o T1: iso to lightly hypointense to brain o T1 C+: vivid enhancement o T2: variable / mixed • MR spectroscopy: cyst contents does not show a broad lipid spectrum as they are filled with water fluid
  15. 15. T1
  16. 16. T1C
  17. 17. T2
  18. 18. • Treatment is usually surgical with radiotherapy especially useful for incomplete resection. • Benign local recurrence is seen in up to a third of patients. o papillary has a much lower recurrence rate than adamantinomatous • Differentials o Rathke’s cleft cyst. o Pituitary macroadenoma o Intracranial terratoma.

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