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
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
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.