Neuro Radiology - Craniopharyngiom

aDr.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 adultso Papillary type

• Similar incidence in males and females.

Clinical presentation:

• Headaches and raised ICP• Visual symptoms

o 20% of children o 80% adults

• Hormonal imbalanceso short stature and delayed puberty in childreno decreased libidoo amenorrhoeao 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 childreno 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 adultso 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 featureo near CSF density

• solid componento soft tissue densityo vivid enhancement

• calcificationo 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 braino T1 C+: vivid enhancemento T2: variable / mixed 

• calcificationo difficult to appreciate on conventional imagingo 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 featureo near CSF density

• solid componento soft tissue densityo vivid enhancement

• calcificationo uncommon - rare

MRI:• cysts

o when present they are variable in signalo 85% T1 hypointense 

• solid componento T1: iso to lightly hypointense to braino T1 C+: vivid enhancemento 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

• Differentialso Rathke’s cleft cyst.o Pituitary macroadenomao Intracranial terratoma.

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