CRANIOPHARYNGIOMA

BY
DR.ROOHIA

craniopharyngioma

Inroduction
Frequency
Pathology
Clinical presentation

introduction

Craniopharyngioma
was the name introduced
by Cushing for tumors
derived from epithelial
rests ascribable to an
imperfect closure of the
hypophysial or
craniopharyngeal duct.
Craniopharyngiomas are
benign tumors that occur
at the base of the brain,
above the pituitary gland.

Craniopharyngioma is a slowgrowing, extra-axial, epithelialsquamous, calcified, cystic tumor.

( WHO grade I)
arising from remnants of the
craniopharyngeal duct and/or Rathke
cleft and occupying the (supra)sellar
region.

Frequency
INCIDENCE:
Annual incidence rate being between 0.5 and 2.5
new cases per million population per year.
Age:
Bimodal- 5-10yr & 50-60yr
Adults- 1-3% of intracranial tumors
- 13-15% sellar or supra sellar
Childrens-56% of sellar or supra sellar tumors.
Sex:
slight male predominance 55%
Race:
Higher in Africa,Far east & Japan(18,16,10.5%)

ETIOLOGY

The embryogenetic theory

The embryogenetic theory relates to the
development of the adenohypophysis and
transformation of the remnant ectoblastic cells
of the craniopharyngeal duct and the involuted
Rathke pouch.
Rathke pouch and the infundibulum develop
during the fourth week of gestation and
together form the hypophysis.
Rathke cleft, together with remnants of the
craniopharyngeal duct, can be the site of origin
of craniopharyngiomas.

1.

2.

3.

The metaplastic theory

The metaplastic theory relates to the
residual squamous epithelium which may
undergo metaplasia.
The dual theory explains the
craniopharyngioma spectrum
adamantinous type (most prevalent in
childhood) to embryonic remnants.
adult type (squamous papillary) to
metaplastic foci derived from mature cells
of the anteriorhypophysis.
Mixed type 15%

MOLECULAR BIOLOGY

Some craniopharyngiomas are

monoclonal in origin, and cytogenetic
abnormalities have been reported in
chromosomes 2 and 12.
Mutations of the -catenin gene have
been identified in 70% of
adamantinomatous craniopharyngiomas.

HISTOLOGY
GROSS: smooth lobulated capsule
Solid- calcified
Cystic-machine oil
(crankcase oil)

ADAMANTINOMATOUS

Epithelial lesion with

peripheral palisading of
basal squamous epithelium
surrounding loosely
arranged epithelial cells,
the so-called "stellate
reticulum" and nodules of
keratin and variable
calcification are typical
histologic features of a
craniopharyngioma.
These nodules are
referred to as "wet" keratin
because of the plump
appearance of the
keratinocytes.

PAPILLOMATOUS TYPE

composed of
simple squamous
epithelium and
fibrovascular
islands of
connective
tissue.

Mixed type

The brain
parenchyma that
surrounds both
variants of
craniopharyngio
ma is typically
gliotic and often
shows profuse
numbers of
eosinophilic
Rosenthal fibers.

Rosenthal fibers in neuropil

surrounding the
craniopharyngioma.

Differences between
Papillary
adamantinomatous
and papillary
craniopharyngiomas
craniopharyngiomas

Less common
Adamantinomatous
craniopharyngiomas

More common
Occur at a younger age
Commonly calcified
Commonly cystic and filled with
cholesterol-rich fluid or soft
necrotic debris.
A palisading layer of basaloid
epithelium surrounds irregularly
arranged cells that resemble the
stellate reticulum of the epidermis.
These nests may be solid but often
form a complex trabecular network
of microcysts. Bands of fibrous
tissue weave between nests of
epithelial cells and around cyst
Keratin nodules are commonly
seen.

Occur at an older age

Calcification is less
common
Commonly solid.
Squamous epithelial
nests that surround
loose fibrovascular
tissue rather than
microcysts create a
solid tumor with a
pseudopapillary
pattern.
Keratin nodules are
not seen.

VASCULAR SUPPLY

A1 segment of the anterior cerebral

artery.
proximal portion of the posterior
communicating artery.
intracavernous meningohypophyseal
arteries.

CLINICAL PRESENTATION

Anterior extension to the

prechiasmatic cistern
and subfrontal spaces;
posterior extension into
the prepontine and
interpeduncular cisterns,
cerebellopontine angle,
third ventricle, posterior
fossa,and foramen
magnum; and laterally
toward the subtemporal
spaces.

headaches and raised ICP

visual symptoms
20% of children
80% adults

hormonal imbalances
short stature and delayed
puberty in children
decreased libido
amenorrhoea
diabetes insipidus

behavioural change due

to frontal or temporal
extension

prechiasmal localization typically results in

associated findings of optic atrophy (eg,
progressive decline of visual acuity and
constriction of visual fields).
retrochiasmal location commonly is
associated with hydrocephalus, with signs of
increased intracranial pressure (eg,
papilledema and horizontal double vision).
intrasellar craniopharyngioma usually
manifests with headache and
endocrinopathy.

Tumour classification

1.
2.
3.
4.

Hoffman et al: sella turcica

optic chiasm
floor of 3rd ventricle
Prechiasmatic
Retrochiasmatic
Subchiasmatic
intraventricular

Sammi et al: vertical projection

Grade I- intra sellar/infra diaphragmatic
Grade II-Occupying cistern with/with out
an
intrasellar component.
Grade III- Lower of 3rd ventricle
Grade IV- Upper of 3rd ventricle
Grade V- Reaching the septum pllucidum
or lateral ventricle.

Differential diagnosis

Rathke's cleft cyst

Pituitary macroadenoma(with cystic degeneration /

necrosis)

no solid / enhancing component

unilocular
majorityare completely or mostly intrasellar

can look very similar

usually hasintrasellarepicentre with pituitary fossa enlargement
rather than suprasellar epicentre
despite occasional presence of T1 bright cystic regions,
calcification in these cases is often absent (whereas
mostadamantinomatouscraniopharyngiomas are calcified)

Intracranial teratoma

presence of fat is helpful, but requires fat saturated sequences or

CT of confirm

THANK YOU