CRANIOPHARYNGIOMA
Follicle-stimulating hormone (FSH)
Other names
 Rathke pouch tumor,
 suprasellar cyst,
 pituitary epidermoid tumor,
 ameloblastoma
 pituitary adamantinoma
Definition
Are histologically benign tumors that are found in the sella
tursica and are composed of squamous epithelial cells.
Origin
The origin of these tumors is presumed to be from rests of
embryonic tissue located in Rathke pouch, an embryonic
structure that later forms the anterior pituitary gland.
Age
 These tumors may appear clinically at any age and
constitute 6 to 10% of all intracranial tumors in children.
 Although craniopharyngiomas usually occur in the
suprasellar region they may extend superiorly into the
third ventricle, or inferiorly along the base of the brain.
 Usual course - progressive.
Morbidity
Although histologically benign, the clinical behavior of this tumour
and its proximity to the optic chiasm, carotid arteries, third
cranial nerve, and pituitary stalk, make this a difficult tumor to
treat without morbidity.
Clinical presentation
 Most often the presenting complaints are secondary to
central-nervous-system involvement by the tumor; they
include headaches, vomiting, visual disturbances, symptoms
of diabetes insipidus, and changes in sensorium.
 Abnormal visual examination, include papilledema (27%),
bitemporal hemianopia (52%), homonymous hemianopia
(8%), unilateral hemianopia (8%), significant unilateral visual
acuity deficit (35%), and bilateral visual acuity deficit
 In some patients there are no signs of endocrine deficiency ;
in others growth arrest and pubertal delay may be obvious
features
 If the tumor invades the hypothalamus, signs of
impaired vegetative function may occur. These include
poikilothermia, hypersomnia, and obesity.
Anatomy
 The most common location of these tumors is a
combination of intrasellar and suprasellar (70%).
 Twenty percent are only suprasellar, and 10% are purely
intrasellar.
 These tumors occasionally grow into the third ventricle,
causing hydrocephalus.
 The arterial supply is usually from the anterior cerebral and
anterior communicating arteries or from the internal carotid
and posterior communicating arteries.
Investigations
Lab Studies:
A full pituitary endocrine workup is mandatory.
1. Corticotropin (ie, adrenocorticotropic hormone [ACTH])
2. Growth hormone (GH) and insulin growth factor (IGF-1)
3. Cortisol
4. Prolactin
5. Luteinizing hormone (LH)
JUDY WAWIRA GICHOYA YR 2007
6.
7. Thyrotropin (ie, thyroid-stimulating hormone [TSH])
8. Triiodothyronine (T3), Thyroxine (T4)
Routine CBC count, chemistry panel, and coagulation studies are
needed before surgery.
Imaging
1. Skull x-ray Because many as 88% of patients have
suprasellar or intrasellar calcification
Even when calcification is not present there is often ballooning
of the sella or erosion of the sella walls and posterior clinoid
processes
2. CT-scan
3. MRI Advantage over CT is that MRI can easily visualize the
tumor in multiple planes and also give the surgeon a better
view of the relationship of the tumor to the arteries of the
Circle of Willis, pituitary stalk, and the optic and oculomotor
nerves.
Management
 Controversy with respect to the extent of surgical resection
and the use of postoperative irradiation
 The ultimate goal is to prevent recurrence of the tumor
with the least impairment of endocrine and intellectual
function.
 These goals can be achieved either by complete surgical
removal of the tumor, or by cyst drainage or partial
resection followed by radiotherapy.
 Surgery is by the trans-sphenoidal approach, in which the
surgeon approaches the sella turcica by a submucosal
incision through the nose along the nasal septum
approaching the pituitary fossa via the sphenoid sinus.
 The pterional craniotomy is the standard approach to
suprasellar lesions because it allows good visualization of
the optic nerves and chiasm.
 Variations of the pterional craniotomy have been proposed
to include resection of the orbital rim and zygoma so as to
provide a more basal view and therefore better access to
the superior aspects of this tumor
 Complete surgical resection will obviate the need for
irradiation, but such aggressive surgery usually results in
permanent endocrine dysfunction and sometimes behavioral
and intellectual abnormalities.
 Radiotherapy usually spares remaining endocrine
dysfunction, but can cause intellectual problems, especially
in the younger child.
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