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Neuro Radiology -
Craniopharyngioma
Dr.Roopchand.PS
Senior Resident Neurology
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
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.
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.
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%
• 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
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.
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.
• 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
• 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
T1
T1C
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
• 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
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
T1
T1C
T2
• 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.
Neuroradiology craniopharyngioma

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Neuroradiology craniopharyngioma

  • 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. 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. 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. 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. • 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. 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. 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. • 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.
  • 11.
  • 12. • 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
  • 13.
  • 15.
  • 16. 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
  • 17. • 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
  • 18.
  • 19. 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
  • 20. T1
  • 21. T1C
  • 22. T2
  • 23. • 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.