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Iran J Radiol 2010, 7(2) 79 NEURORADIOLOGY Imaging of Monstrous Craniopharyngioma: A Pictorial Essay Craniopharyngioma is usually a mixed solid and cystic suprasellar tumor, which usually occurs in children and adults in the fourth and fifth decades of life. The tumors are histologically benign and slow growing, but focal invasion and peritumoral fibrosis leads to high tumoral recurrence after surgery. We had 11 patients admitted with different symptoms such as headache, nausea, vomiting and visual loss in whom extensive craniopharyngioma was proved after surgery. In some patients, primary imaging studies were reviewed. In others who presented with new onset symptoms several years after surgery, brain CT scan and MRI studies were performed. On brain CT scan of most cases, a mixed cystic and solid suprasellar mass with calcifications in both solid and cystic parts was detected. In this article, we present some cases of large cra- niopharyngioma with interesting extensions and invasions to adjacent brain structures. Keywords: Craniopharyngioma, Computed Tomography, Magnetic Reson- ance Imaging Introduction raniopharyngioma is a histologically benign slow-growing tumor (WHO grade Ι), 1,2 which accounts for approximately 3% of all intracranial tumors. 3,4 It has a bimodal age distribution with the first peak in children and the second in adults at age 40-60 years. It is not predominant in any sex or race. 1 It originates from the embryonic precursor of adenohypophysis which is called Rathke’s pouch. 1,5,6 The adenohypophysis and infandibulum migrate from the pharynx to the sella turcica during fetal life. This fact shows that craniopharyn- giomas may occur anywhere along this migration tract. 1 Craniopharyngioma has three histological types: adamantinomatous, papillary and mixed. 1 The adamantinomatous type predominantly occurs in children. They are classically cystic tumors with a solid component. 1,2,7 The fluid in the cyst is usually similar to motor oil. 8,9 It contains high protein materials, choles- terol crystals and blood products. The solid part contains calcification, squamous or columnar epithelium, fibrosis and inflammation. Complete resection of the tumor is difficult because of this fibrosis and dense adhesion to adjacent struc- tures. This issue leads to frequent recurrence of tumor in patients treated with surgery. The papillary type predominantly occurs in adults. It has only a solid component typically without calcification. 1,7 This type is more encapsulated and has less recurrence after surgical resection. 1 Because of the slow-growing nature of tumors, the symptoms develop insi- diously and the common interval between onset of symptoms and diagnosis is 1- 2 years. 2 The most frequent reported symptoms are headache, nausea, vomiting and visual disturbance. Growth failure and hypogonadism are also encountered frequently. Bitemporal hemianopia is the most frequent disturbance of the visual C H.R. Haghighatkhah MD 1 M. Sanei Taheri MD 1 M. Haghighi MD 2 S. Shahzadi MD 3 Sh. Birang MD 4 1. Associate Professor, Department of Radiology, Shohada Tajrish Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran. 2. Resident of Radiology, Shohada Tajrish Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran. 3. Professor of Neurosurgery, Shohada- Tajrish Hospital, Shahid Beheshti Uni- versity of Medical Sciences, Tehran, Iran. 4. Associate Professor, Department of Radiology, Loghman Hospital, Shahid- Beheshti University of Medical Sciences, Tehran, Iran. Corresponding Author Shirin Birang Department of Radiology, Loghman Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran. Tel: +9821 5541 1411 Fax: +9821 5541 1411 E-mail: [email protected] Received September 2, 2009; Accepted after revision March 8, 2010 Iran J Radiol 2010;7(2):79-89

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Iran J Radiol 2010, 7(2) 79

NEURORADIOLOGY

Imaging of Monstrous Craniopharyngioma: A Pictorial Essay Craniopharyngioma is usually a mixed solid and cystic suprasellar tumor, which usually occurs in children and adults in the fourth and fifth decades of life. The tumors are histologically benign and slow growing, but focal invasion and peritumoral fibrosis leads to high tumoral recurrence after surgery. We had 11 patients admitted with different symptoms such as headache, nausea, vomiting and visual loss in whom extensive craniopharyngioma was proved after surgery. In some patients, primary imaging studies were reviewed. In others who presented with new onset symptoms several years after surgery, brain CT scan and MRI studies were performed. On brain CT scan of most cases, a mixed cystic and solid suprasellar mass with calcifications in both solid and cystic parts was detected. In this article, we present some cases of large cra-niopharyngioma with interesting extensions and invasions to adjacent brain structures.

Keywords: Craniopharyngioma, Computed Tomography, Magnetic Reson-ance Imaging

Introduction

raniopharyngioma is a histologically benign slow-growing tumor (WHO

grade Ι),1,2 which accounts for approximately 3% of all intracranial tumors.3,4

It has a bimodal age distribution with the first peak in children and the second in

adults at age 40-60 years. It is not predominant in any sex or race.1

It originates from the embryonic precursor of adenohypophysis which is called

Rathke’s pouch.1,5,6 The adenohypophysis and infandibulum migrate from the

pharynx to the sella turcica during fetal life. This fact shows that craniopharyn-

giomas may occur anywhere along this migration tract.1

Craniopharyngioma has three histological types: adamantinomatous, papillary

and mixed.1 The adamantinomatous type predominantly occurs in children.

They are classically cystic tumors with a solid component.1,2,7 The fluid in the

cyst is usually similar to motor oil.8,9 It contains high protein materials, choles-

terol crystals and blood products. The solid part contains calcification, squamous

or columnar epithelium, fibrosis and inflammation. Complete resection of the

tumor is difficult because of this fibrosis and dense adhesion to adjacent struc-

tures. This issue leads to frequent recurrence of tumor in patients treated with

surgery. The papillary type predominantly occurs in adults. It has only a solid

component typically without calcification.1,7 This type is more encapsulated and

has less recurrence after surgical resection.1

Because of the slow-growing nature of tumors, the symptoms develop insi-

diously and the common interval between onset of symptoms and diagnosis is 1-

2 years.2 The most frequent reported symptoms are headache, nausea, vomiting

and visual disturbance. Growth failure and hypogonadism are also encountered

frequently. Bitemporal hemianopia is the most frequent disturbance of the visual

C

H.R. Haghighatkhah MD1 M. Sanei Taheri MD1 M. Haghighi MD2 S. Shahzadi MD3 Sh. Birang MD4

1. Associate Professor, Department of Radiology, Shohada Tajrish Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran. 2. Resident of Radiology, Shohada Tajrish Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran. 3. Professor of Neurosurgery, Shohada-Tajrish Hospital, Shahid Beheshti Uni-versity of Medical Sciences, Tehran, Iran. 4. Associate Professor, Department of Radiology, Loghman Hospital, Shahid-Beheshti University of Medical Sciences, Tehran, Iran. Corresponding Author Shirin Birang Department of Radiology, Loghman Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran. Tel: +9821 5541 1411 Fax: +9821 5541 1411 E-mail: [email protected] Received September 2, 2009; Accepted after revision March 8, 2010 Iran J Radiol 2010;7(2):79-89

Haghighatkhah et al.

80 Iran J Radiol 2010, 7(2)

field. Other presentations include seizures, preco-

cious puberty, inappropriate secretion of ADH, hear-

ing loss and epistaxis.10

In patients with raised intracranial pressure or rapid

visual function deterioration, we must focus on sur-

gical treatment of hydrocephalus or tumor cyst de-

compression. The main management of tumor is sur-

gery with an attempt to gross total resection of tu-

mor, but in cases with extensive tumors in which to-

tal resection is impossible, limited resection with tu-

moral debulking is recommended to reduce the tu-

moral compression of optic pathways and to help CSF

drainage. Limited surgical resections are followed by

radiotherapy.2

One of the most interesting issues in craniopharyn-

gioma imaging is its extension and invasion to mul-

tiple brain structures, which is best appreciated on

MRI. These tumors have different patterns of exten-

sion such as superior extension from the suprasellar

region with compression of the third or lateral ven-

tricles, lateral extension to parasellar areas, cavernous

sinuses and temporal lobes, anterior extension to

frontal lobes with superior displacement of A1 seg-

ment of the anterior cerebral artery and anterior

communicating artery, posterior extension to perime-

sencephalic cisterns, brain stem, compression of the

basilar trunk and involvement of cerebellum and su-

perior cerebellar cistern and finally inferior extension

to sella, sphenoid and ethmoidal sinuses and nasopha-

rynx. Identifying these patterns of extension can help

us in the surgical management of craniopharyngi-

omas to decide whether total or partial resection is

recommended and to differentiate these tumors from

other extensive skull-base tumoral lesions.

We encountered 11 patients with surgically con-

firmed craniopharyngioma. These are extensive tu-

mors with unusual extension to their neighboring

brain structures. Their brain CT scan and MR images

are reviewed.

Case Presentation

Patient 1

The first patient is a six-year-old boy who presented

with nausea, vomiting and ataxia 2 years after surgic-

al treatment of craniopharyngioma. Brain CT scan

showed a tumoral lesion in the suprasellar region

with extension to the middle and posterior cranial

fossa. The tumor is predominantly cystic with solid

components. Eggshell calcification is seen in the cys-

tic part. The solid part is also calcified in both pop-

corn and solid lump appearances. Right temporal cra-

niotomy and an intracystic catheter are noted as well.

On brain MRI study, the mass is seen with both sol-

id and cystic parts. The cystic part is hypointense on

T1W and hyperintense on T2W images. After con-

trast injection, homogeneous enhancement is seen in

the solid part and rim enhancement is also seen in the

cystic part. The tumor extends from the suprasellar

region to sella and then from the perimesencephalic

cisterns to the posterior fossa, superior cerebellar cis-

tern and cerebellum. It also extended to the right lat-

eral ventricle and third ventricle and produces biven-

tricular hydrocephalus due to compression of fora-

men of Monro. Pathologic examination revealed an

adamantinomatous craniopharyngioma (Fig. 1).

Patient 2

The second patient is an 11-year-old boy who pre-

sented with visual loss 1.5 years ago. Brain CT scan

revealed a tumoral lesion with both solid and cystic

parts in the suprasellar and sellar regions. Calcifica-

tion is seen in the lesion, which has eggshell configu-

ration in the cystic part and lumpy appearance is also

seen in the solid part. The tumor extends to the post-

erior cranial fossa. It also extends to the third ven-

tricle from its superior margin. On MRI study, a T1

isointense and T2 hyperintense cystic lesion is seen in

the suprasellar region with extension to sella turcica

and involvement of the left parasellar region with left

carotid artery encasement and left cavernous sinus

invasion. The tumor extended to the posterior fossa

and compressed the brain stem at its posterior mar-

gin. It deviated the pons, midbrain and fourth ven-

tricle to the right. In addition, it compressed the basi-

lar trunk at its posterior margin. At the superior mar-

gin, the tumor extended to the third ventricle and

produced biventricular hydrocephalus. After contrast

injection, thin rim enhancement was seen in the cys-

tic component and nodular enhancement was en-

countered in the solid part (Fig. 2).

Monstrous Craniopharyngioma: A Pictorial Essay

Iran J Radiol 2010, 7(2) 81

Patient 3

We encountered a six-year-old girl with headache,

nausea and vomiting from 6 months ago. CT scan of

the brain showed a mixed solid and cystic lesion with

heavy calcification in the solid and cystic parts. The

cystic part was seen with hypodense cystic contents.

The tumor extended to the right lateral and third

ventricles and produces biventricular hydrocephalus.

On the MR imaging study, a mixed cystic solid tumor

with low signal cystic contents on T1W was seen in

the suprasellar region with extension to the right tha-

lamus and lateral ventricles. Thick rim enhancement

was seen in the wall of the cystic part after contrast

injection. The tumor was adamantinomatous in pa-

thologic examination (Fig. 3).

Patient 4

The fourth patient is a 12-year-old girl who pre-

sented with left eye blindness from 5 months ago. On

brain CT scan performed after stereotaxis and placing

an intracystic catheter, a heavily calcified suprasellar

mass with extension to the sella was seen. Erosion

and destruction of the anterior and posterior clinoid

processes were noted. The mass extended to the ante-

rior and posterior cranial fossa. A large cystic compo-

Fig. 1. A six-year-old boy with nausea, vomiting and ataxia 2 years after surgical treatment of craniopharyngioma. A. Axial contrast enhanced CT scan of the brain, supra and parasellar cystic tumor with rim enhancement, extension to the posterior fossa and encasement of the basilar artery. B. Axial contrast enhanced CT scan of the brain, cystic and solid tumor with calcification and enhancement of solid and cystic parts, tumoral extension to the third ventricle and posterior fossa is seen. C. Axial contrast enhanced CT scan of the brain, extension of craniopharyngioma to the su-pratentorial region with compression of Monro's foramen. Drainage catheter is seen in the cystic part. D. Sagittal T1W MRI with contrast, low-signal cystic tumor with rim enhancement, extension to prepontine cistern and invasion to the midbrain and cerebellum. E. Sagittal T1W MRI with contrast, tumoral extension from perimesencephalic cisterns to the posteriorfossa and supratentorial area with biventricular hydrocephalus induction. F. Coronal T1W MRI with contrast, tumoral extension to cerebellum and lateral ventricles. G. Coronal T2W MRI, high signal cystic tumor with compression of the third ventricle.

A B C

D E F

G

Haghighatkhah et al.

82 Iran J Radiol 2010, 7(2)

nent was seen in the left frontal region with mass ef-

fect and midline shift to the right. The MRI study

was performed one year after surgery, the tumoral

remnant was seen in the left thalamus and deep tem-

poral lobe which has low signal intensity on T1W

and high signal intensity on T2W images. Nodular

enhancement was seen in the periphery of the cystic

part after contrast injection. Pathologic examination

Fig. 2. An 11-year-old boy with visual loss from 1.5 years ago. A. Axial brain CT scan, suprasellar lesion with calcification of the solid and cystic parts and extension to the posterior fossa. B. Axial brain CT scan, tumoral extension to the third ventricle and compression of the foramen of Monro. C. Axial T1W MRI, tumoral extension to the left parasellar area and left temporal lobe. Invasion to the posterior fossa and brainstem is noted as well. D. Coronal T2W MRI, tumoral extension to the left parasellar area and encasement of the left carotid artery.

Fig. 3. A six-year-old girl with headache, nausea and vomiting from 6 months ago. A. Axial brain CT scan, heavily calcified tumor with extension to the third ventricle and biventricular hydrocephalus induction. B. Axial brain CT scan, cystic and calcified tumor with compression of the right lateral ventricle and midline shift to the left. C. Axial T1W MRI, low signal cystic lesion with involvement of the right tha-lamus. D. Sagittal T1W MRI with contrast, thick rim enhancement of the lesion.

D

B C

B CA

A

D

Monstrous Craniopharyngioma: A Pictorial Essay

Iran J Radiol 2010, 7(2) 83

revealed adamantinomatous craniopharyngioma (Fig.

4).

Patient 5

A 17-year-old female was admitted with headache

and epistaxis from one year ago. Seven years ago she

was operated because of craniopharyngioma. Brain

CT scan showed a heavily calcified mixed solid and

cystic mass in the suprasellar area with extension to

the right cerebellopontine angle and third ventricle.

Brain MRI revealed a mixed low and high signal in-

tensity mass on T2W images in the suprasellar area

with extension of cystic part to left temporal lobe and

right cerebellopontine angle and interpeduncular cis-

terns. It is extended to anterior part of midbrain and

cerebral peduncles. Low signal areas are seen in tu-

moral mass due to calcification. After contrast injec-

tion, the solid tumoral portion was enhanced densely

and homogeneously. Comprehension of the basilar

trunk was seen in the posterior aspect of the tumor as

well as superior displacement of A1 segment of ante-

rior cerebral artery (ACA) (Fig. 5).

Patient 6

A 15-year-old boy was admitted to hospital with

headache and bitemporal hemianopia from 14

months ago. Brain CT scan study was performed 3

years after surgery. A heterogeneous calcified cystic

and solid mass was seen in the sellar and suprasellar

regions with extension to the anterior and posterior

cranial fossae and also to sphenoidal and posterior

ethmoidal sinuses and nasopharynx. Anterior and

posterior clinoid processes, sellar floor and sphenoidal

and ethmoidal bones are eroded. At the superior mar-

gin, the mass extended to the interpeduncular cistern.

A hypodense area is seen in the right frontal lobe in

favor of post-surgical encephalomalacic changes. A

drainage device is also seen in the cystic part of the

mass. Brain MRI revealed an isointense mass with

gray matter on T1W images with nodular and rim

enhancement and low signal areas in favor of calcifi-

cation. The mass extended to the nasopharynx, post-

erior ethmoidal, left maxillary and sphenoidal sinuses

from its inferior margin. It also extended to the post-

erior fossa and compresses the brain stem and the ba-

silar trunk. From the superior margin, it extended to

the interpeduncular cistern. Deviation of both inter-

nal carotid arteries and invasion of both cavernous

sinuses are noted as well. A porencephalic area in the

right frontal lobe and the drainage catheter are seen

on MRI and CT scan. Pathologic examination re-

vealed an adamantinomatous craniopharyngioma

(Fig. 6).

Patient 7

A 5-year-old girl presented with visual loss 3 years

after surgical treatment of craniopharyngioma. A cal-

cified mixed solid and cystic mass was seen in the su-

prasellar area on brain CT scan. The tumor extended

to the third ventricle and posterior fossa in the right

cerebellopontine angle. Biventricular hydrocephalus

was also noted. On T1W brain MRI study, right tem-

poroparietal craniotomy is noted. A mixed solid-

cystic mass is seen in the suprasellar area with exten-

sion to the third ventricle and compression of lateral

ventricles with hydrocephalus induction in the right

lateral ventricle. Low signal cystic materials are noted

on T1W images. The solid part is seen with high sig-

nal intensity in some parts on T1W images probably

due to calcification. A multi-cystic mass with low

signal areas of calcification is seen on T2W images as

well. Encasement of the proximal segment of the

middle cerebral artery (MCA), ACA, the distal part of

the internal carotid artery (ICA) and the basilar trunk

is noted. Invasion to the brain stem from the post-

erior tumoral margin is noted as well (Fig. 7).

Patient 8

A six-year-old girl was admitted with headache,

nausea and vomiting 3 years after surgical treatment

of craniopharyngioma. Physical exam revealed para-

lysis of lateral gaze in the left eye. Brain CT scan

showed a mixed solid and cystic mass with calcifica-

tion in the suprasellar region with extension to the

left temporal lobe and anterior cisterns of the brain

stem. On MRI study, a T1Wand T2W high signal in-

tensity cystic mass is seen in the suprasellar region

with extension to the left parasellar area and encase-

ment of the left ICA and invasion to the left cavern-

ous sinus and both temporal lobes. Compression and

right deviation of the third ventricle are noted as

well. Midline shift to the right is seen as well as supe-

rior displacement at A1 segment of the ACA.

Haghighatkhah et al.

84 Iran J Radiol 2010, 7(2)

Patient 9

A 23-year-old woman presented with headache,

visual loss, nausea and vomiting 6 years after surgical

treatment of craniopharyngioma. Brain MRI study

was performed. A multi cystic mass with low signal

intensity on T1W and high signal intensity on T2W

images is seen in the sellar and suprasellar regions.

Thin rim enhancement of the cystic part and nodular

enhancement of the solid part are also noted after

contrast injection. The mass extends to the right fron-

tal lobe and compressed the optic chiasm. Compres-

sion of the third ventricular floor is also noted. Pre-

vious craniotomy scar is seen in the left parietal re-

gion (Fig. 9).

Patient 10

A six-year-old boy was admitted with headache,

Fig. 4. A 12-year-old girl with left eye blindness from 5 months ago. A. Axial brain CT scan, heavily calcified suprasellar mass with extention to both frontal lobes and posterior fossa. B. Axial brain CT scan, large cystic tumoral part in the left frontal lobe with mass effect and midline shift to the right. C. Sagittal T1W MRI, tumoral recurrence 2 years after operation. D. Sagittal T1W MRI with contrast, nodular and rim enhancement is seen in solid and cystic parts.

Fig. 5. A 17-year-old female with headache and epistaxis from one year ago who was admitted 7 years after surgical treatment of craniopharyngioma. A. Axial brain CT scan, heavily calcified suprasellar mass with extension to the right CP angle. B. Axial brain CT scan, tumoral invasion to the posterior aspect of the third ventricle. C. Axial T2W MRI, mixed solid and cystic tumor of suprasella with low signal areas of calcification, exten-sion to the left temporal lobe and anterior aspect of the midbrain. D. Sagittal T1W MRI, high signal tumor with superior displacement of the A1 segment of ACA. Craniotomy defect is seen at the parietal area. E. Sagittal T1W MRI, tumoral invasion to prepontine cistern and compression of the basilar trunk.

A B C D

E

A B

DC

Monstrous Craniopharyngioma: A Pictorial Essay

Iran J Radiol 2010, 7(2) 85

nausea, vomiting and ataxia from 1.5 years ago. Brain

MRI study was performed. An extensive solid and

cystic mass is seen in the anterior, middle and post-

erior cranial fossae. The mass had multi-cystic com-

ponents with high and low signal contents on T1W

images. The high signal cystic part extends to the an-

terior fossa and compresses both frontal lobes and the

optic chiasm. T1W imaging shows a low signal cystic

part extending to the third ventricle with compres-

sion of the foramen of Monro and lateral ventricular

hydrocephalus induction. Reticular and non homo-

geneous enhancement is seen in the solid parts. Right

and left parasellar extension is noted as well. Post-

erior extension to the prepontine and interpeduncu-

Fig. 6. A 15-year-old boy with headache and bitemporal hemianopia from 14 months ago. A. Axial brain CT scan, suprasellar tumoral mass with calcification and invasion to the sphenoidal and posterior ethmoidal sinuses. Extension to posterior fossa is noted as well. B. Axial contrast enhanced brain CT scan, cystic mass with eggshell calcification and extension to frontal lobes. The drainage catheter and right frontal encephalomala-ciais seen. C. Axial T1W MRI with contrast, nonhomogeneous en-hancing mass with invasion to the posterior fossa, both parasellar areas and posterior ethmoidal sinuses.

Fig. 7. A five-year-old girl with visual loss 3 years after surgical treatment of craniopharyngioma. A. Axial brain CT scan, solid and cystic calcified suprasellar mass with extension to the right CP an-gle and compression of the right side of pons. B. Axial brain CT scan, tumoral extension to the third ventricle with biventricular hydrocephalus induc-tion. C. Coronal T1W MRI with contrast, low signal cystic mass with high signal solid parts due to calcifica-tion. Mass effect, midline shift and right lateral ventricular hydrocephalus is evident. D. Sagittal T2W MRI, high signal multicystic mass with low signal areas of calcification. Invasion to the brainstem and encasement of the arteries of the circle of Willis is evident.

D. Axial T1W MRI, tumoral extension to both parasellar areas and encasement of the internal carotid arteries, invasion to both cavernous sinuses is observed. E. Axial T1W MRI with contrast, tumoral invasion to both frontal lobes and compression of anterior aspect of the midbrain.

D E

A B C

A B C

D

Haghighatkhah et al.

86 Iran J Radiol 2010, 7(2)

lar cisterns with basilar trunk compression is pre-

sented (Fig. 10).

Patient 11

A six-year-old boy presented with testicular en-

largement from 4 years ago. Brain CT scan was per-

formed and a hypodense cystic suprasellar mass with

superior extension to the third ventricle is noted.

Thin eggshell calcification is also seen in the walls of

the cystic part. Biventricular hydrocephalus is de-

tected. On MRI study, a T1W low signal cystic mass

with iso signal solid parts is seen in the suprasellar

region with superior extension to the third ventricle

and inferior extension to sella turcica. Encasement of

the left ICA bifurcation and superior displacement at

M1 segment of the MCA is noted. Posterior extension

to the midbrain and basilar trunk compression is seen

as well. Biventricular hydrocephalus is evident on

MRI and CT scan (Fig. 11).

Fig. 8. A six-year-old girl with headache, nausea and vomiting 3 years after surgical treatment of cra-niopharyngioma. A. Axial contrast enhanced brain CT scan, mixed solid and cystic suprasellar mass with calcification and extension to the left temporal lobe. B. Axial T1W MRI, high signal suprasellar mass with extension to the left frontal and temporal lobes. C. Axial T2W MRI, high signal cystic mass with extension to the left temporal lobe. D. Coronal T1W MRI, tumoral extension to the left parasellar region with left ICA encasement and left cavernous sinus invasion.

Fig. 9. A 23-year-old female with headache, visual loss, nausea and vomiting 6 years after surgical treatment of craniophryngioma. A. Coronal T1W MRI with contrast, low signal suprasellar cystic mass with nodular enhancement of the solid part and compression of the third ventricle. B. Sagittal T1W MRI with contrast, tumoral extension to the anterior fossa. C. Axial T2W MRI, high signal cystic tumor with extension to both frontal lobes.

A B

D

A B C

C

Monstrous Craniopharyngioma: A Pictorial Essay

Iran J Radiol 2010, 7(2) 87

Discussion

Craniopharyngioma is the most common pediatric

intracranial tumor of non-glial origin. This tumor

should be considered in the differential diagnosis of

suprasellar, parasellar, prepontine and posterior fossa

tumors. Rathke cleft cyst, suprasellar, arachnoid cyst,

hypothalamic or chiasmatic astrocytoma, and even

dermoid or epidermoid cysts may be mimicked by

some kinds of extended craniopharyngiomas at imag-

ing. Being familiar with unusual and interesting im-

aging presentations of these common pediatric tu-

mors could lead radiologists to a better diagnosis. Craniopharyngiomas are partly cystic, benign, slow

growing, usually suprasellar tumors which account

Fig. 10. A six-year-old boy with headache, nausea, vomiting and ataxia from 1.5 years ago. A. Sagittal T1W MRI with contrast, high and low signal multicystic suprasellar mass with rim enhancement in the cystic part. Involve-ment of frontal lobes and third ventricle is seen. B. Coronal T1W MRI with contrast, tumoral invasion to the third ventricle and lateral ventricular hydrocephalus induction.

Fig. 11. A six-year-old boy with testicular enlargement from 4 years ago. A. Axial brain CT scan, cystic suprasellar mass with third ventricular extension and biventricular hydrocephalus induction. Extension to the posterior fossa and brainstem compression is noted as well. B. Axial T1W MRI, low signal suprasellar cystic mass. C. Axial T1W MRI, third ventricular involvement is noted. D. Sagittal T2W MRI, high signal sellar and suprasellar mass with superior displacement of the M1 segment of MCA.

C D

A B

A B

Haghighatkhah et al.

88 Iran J Radiol 2010, 7(2)

for 5-13% of all intracranial tumors.11 They derive

from Rathke’s pouch, the embryonic infolding of en-

doderm that extends superiorly from the stomodeum

and gives rise in the early fetal development to the

adenohypophysis;7 with manifestation during child-

hood and fourth or fifth decades of adult life. Fre-

quent symptoms of manifestation are visual symp-

toms or endocrine disturbances such as polyuria and

polydipsia due to diabetes insipidus.12 The tumor has

three histologic types: adamantinomatous (pediatric

type), papillary (adult type) and mixed.1

The adamantinomatous type is classically a cystic

tumor with solid components.1,2,5 The papillary type is

predominantly solid, without calcification.1,7 After

surgery and radiation, the 5-year survival rate is more

than 80% in children. Permanent endocrinologic def-

icits are seen in many patients after treatment but

visual disturbances are usually reversible. The recur-

rence rate of the tumor depends on the size of the

tumor, the recurrence rate is approximately 20% for

tumors smaller than 3 cm and 83% for tumors larger

than 5 cm.1

CT and MRI are complementary studies for detec-

tion of extent and characteristics of the tumor.1 On

CT scan, the pediatric type is presented as a predomi-

nantly suprasellar and calcified cystic mass with solid

components in 90% of cases. Extension to sella turci-

ca is seen frequently. The adult type tumors are

usually solid and isodense. Calcifications and intra-

ventricular craniopharyngiomas are rare.

In the papillary subtype, after contrast injection,

enhancement is seen in solid components and the

wall of cystic parts in 90% of cases. Anterior and su-

perior extension of the tumor leads to anterior dislo-

cation of the optic chiasm and superior dislocation of

the A1 segment of the anterior cerebral artery. On

CT scan, the usual extension of the cystic part is to

the anterior and lateral but the solid part extends to

the posterior and lateral.13

MRI is preferred for better determination of the

tumor extent. The pediatric type appears as a predo-

minantly cystic mass with solid components but the

papillary type is predominantly solid.1,7 Calcifications

are better seen in GRE images and may show suscep-

tibility effects. Signal intensity of the cystic part is

variable on T1W images. Blood products, high pro-

tein concentration or cholesterol crystals in the cyst

leads to high signal characteristics of the cystic part

on T1W images. Cysts are hyperintense on T2W and

FLAIR images. Solid components appear isointense

on T1W and heterogeneously iso to hypointense on

T2W images.

A prominent lipid spectrum is seen in MR spectros-

copy around 1PPM because of the cystic contents.

Also variable signal intensities are seen in diffusion

weighted images because of variable cystic contents.

After contrast injection, patterns of enhancement in

solid and cystic tumoral parts are similar to CT scan.13

The location of the tumor is predominantly supra-

sellar in 75%, supra and infrasellar in 12%, and pure-

ly intrasellar in 4% of cases. Encasement or displace-

ment of the vessels forming the circle of Willis is

noted. Lateral displacement of ICAs and superior dis-

location of ACA are also seen. Posterior dislocation

and compression of the basilar trunk is noted as well.

We can categorize the tumoral extension to three

specific subtypes. In type A, the tumors are entirely

within the sella and vascular displacement is not

seen. In type B, the tumor is extended anteriorly be-

tween the optic nerves and pushes the optic chiasm

posteriorly. Elevation of the anterior communicating

artery and the A1 segment of the anterior cerebral

artery are noted as well. In type C, elevation of the

A1 segment of the anterior cerebral artery and ante-

rior communicating artery, with posterior compres-

sion of the basilar trunk is noted. Anterior dislocation

of the optic chiasm with third ventricular compres-

sion and hydrocephalus are seen as well.1

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