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Suprasellar MassDarbi Invergo, DO
September 3, 2010
What comes to mind?
S- Sarcoidosis
A- Adenoma/ Aneurysm/ Arachnoid Cyst
T- Teratoma
C- Craniopharyngioma (v Rathke)/ Cephalocele
H- Hypothalamic Glioma; Hamartoma/ Histiocytosis X/
Hemachromatosis/ Hypertrophy
M- Meningioma/ Mets/ Meningitis (TB)
O- Optic Nerve Glioma
L- Lymphocytic Hypophysitis/ Lipoma/ Lymphoma/ Leukemia
E- Epidermoid (v Dermoid)
Non Contrast CT Contrast Enhanced CT
T2* T1 Pre and Post Contrast
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Nodule, Pre and Post Contrast Normal Caliber of the Carotids
Anteriorly Located ACA
Review the Imaging Characteristics
Calcifications
Intra-Axial Tumors:
Oligodendroglioma (90%)
Ependymoma (50%)
Ganglioglioma (40%)
Ch id Pl P ill (25%)Choroid Plexus Papilloma (25%)
Astrocytoma (20%)
Metastasis
Extra-Axial Tumors:
Craniopharyngioma (90%)
Meningioma (25%)
Chordoma
Chondrosarcoma
High Intensity on T1
Methemoglobin•Pituitary Apoplexy•Hemorrhagic Mass•Thrombosed Aneurysm
High Protein•Proteinaceous Cysts•Neurenteric CystHigh Protein Neurenteric Cyst•Dermoid Cyst
Fat •Lipoma•Dermoid Cyst
Cholesterol •Colloid Cyst
Melanin •Melanoma
Flow Effects •Slow Flow
Paramagnetic Cations •Cu, Mn…
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Low Intensity on T2
Hypercellularity LymphomaMeningiomaPNETGerminomaGBMOligodendrogliomaMucinous AdenoCA mets
Calcification Calcium
Blood Old Blood
Protein Colloid Cyst
Melanin Melanoma
Flow Void HemangioblastomaVascular Malformation
Appears Cystic…Differential for Cystic Sellar
Masses
Craniopharyngioma
Rathke Cleft Cyst
Colloid Cyst
Arachnoid Cyst
Cystic Pituitary Adenoma
Xanthogranuloma
Epidermoid Cyst
Dermoid Cyst
Ependymal Cyst
Gabriel Zada, M.D.,Ning Lin, M.D., Eric Ojerholm, B.S.E., Shakti Ramkissoon , M.D., Ph.D., Edward R. Laws, M.D. Craniopharyngioma and other cystic epithelial lesions of the sellar region: a review of clinical, imaging, and histopathological relationships. Neurosurg Focus 28 (4):E4, 2010
Embryologic Development
Rathke’s pouch
4th week of Embryologic Development
Rostral Outpouching from Roof of Primitive Oral Cavity
Anterior Wall of the Pouch Anterior Lobe of the Pituitary (Pars Distalis)
Posterior Wall of the Pouch DOES NOT Proliferate
I t di t L b f th Pit it (P I t di )Intermediate Lobe of the Pituitary (Pars Intermedia)
Lumen of the Pouch Narrows to form a Cleft
Rathke’s Cleft Normally Regresses
Persistence of this Cleft with Expansion Rathke’s cleft cyst
Cyst wall lined by single columnar cell layer of epithelium, often containing goblet cells, and is often ciliated
Embryologic Development
Two hypotheses: 1.Development of the Adenohypophysis During
Embryogenesis Consistent with Pediatric Tumor Development
Although ~1/3 of all craniopharyngiomas occur in children, the percentage of embryonal childhood tumors ranges from 75 to virtually 100%.
2.Residual Squamous Epithelium from the Adenohypophysis Undergoes Metaplasia Adult Tumors
No Consensus
Adenohypohysis:•Pars distalis - the largest section•Pars tuberalis - a collar of tissue that
usually surrounds the infundibular stalk•Pars intermedia - a narrow band that is
usually separated from the pars distalisby a hypophyseal cleft
Neurohypohysis:•Pars nervosa - the bulk of the
posterior pituitary•Median emminence - the upper
section of the neurohypophysisabove the pars tuberalis
•Infundibular stalk - the "stem" that connects the pars nervosa to the base of the brain
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Rathke's Cleft Cysts
Rathke's Cleft Epithelium
Sellar (40%)/ Suprasellar (60%)
Asymptomatic
13 - 22% of Autopsies
Fluid-Filled with very Thin Walls (only 1-2 cell layers thick)
Secrete Fluid Grow /Compress Adjacent Structures
Rathke's Cleft Cysts
CT
Non contrast : typically non-calcified w/ homogenous low attenuation
Uncommonly it may be of mixed iso- and low attenuation, or contain small curvilinear calcifications in the wall (seen in 10 - 15% of cases)
Post contrast : Typically non enhancing although the cyst wall may enhance in some cases
MRIThe signal characteristics vary according to the cyst composition which may be mucoid or serous
T1 : ~ 50% are hyper intense (protein contents) and 50% are hypo intense.
T2 : ~ 70% are hyper intense and 30% are iso or hypo intense.
T1 C+ (GAD) : no contrast enhancement of the cyst is seen, however a thin enhancing rim of surrounding compressed pituitary tissue may be apparent
70 - 80% of cases a small non-enhancing intracystic nodule can be identified which is virtually pathognomic of a Rathke’s cleft cyst.
When seen it is hyper intense to surrounding fluid on T1 and hypointense on T2
Occasionally a fluid - fluid level may be seen (particularly if there has been a haemorrhage)
CraniopharyngiomaRathke's Cleft Epithelium
Sellar (15%)/ Suprasellar (85%, Anywhere Along the Infundibulum)
Enlarged Sella
Rare / Ectopic LocationsFloor of 3rd Ventricle, Nasopharynx, Posterior Fossa, Extension Down the Cervical SpineDown the Cervical Spine
“Benign” Tumor (WHO Grade I)
Locally Invasive
Symptomatic Hydrocephalus & Endocrine Disturbances
~3% Of All Intracranial TumorsPediatric (5-14 yrs) ~6-13%
Adult (65-74 yrs)
Thick Walls
Complex Mass with Multiple Nodules at the Base of the Brain, Sinuating Along the Fissures
Craniopharyngioma
Adamantinomatous
Predominantly in Children
Reticular Epithelial Cells Resemble Enamel Pulp of
Papillary
Adults
Metaplastic Squamous Cells
Keratin Pearls, No AnuclearResemble Enamel Pulp of Developing Teeth
Cysts Filled with Thick Oily Fluid High in Protein, Blood Products, and/or Cholesterol "Machinery Oil"
"Wet Keratin Nodules"
Calcification Usually Present ~ 90%
β-catenin gene mutations
,Ghost Cells, Encapsulated, Well Differentiated Solid Sheet Like Cells
Stains Positive for Cytokeratin and EMA
Solid > Cystic
No "Wet Keratin"
Calcification Uncommon
No β-catenin gene mutations
Craniopharyngioma
CTHeterogeneous Mass /Suprasellar Region
Calcification (Except Papillary Type) / Cysts Very Common:
Calcification
Overall 80 - 87% Of Craniopharyngiomas Calcified
Adamantinomatous : 90%
Papillary : Rare
Cysts : Seen in 70 - 75% Of Cases (More Frequently in Adamantinomatous Type)
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Craniopharyngioma
MRI
T1 :
Classic Adamantinomatous Type:
Signal Intensity Varies Depending on Cyst Contents, And
± Hyper Intense Due To Protein, Blood Products, &/or Cholesterol
Papillary Type:
Solid Components Appear Iso-Intense
Contrast Enhanced T1:
Thin Enhancement of the Cyst Wall
Diffuse Heterogeneous Enhancement of the Solid Components
T2 : Signal is High in Both Solid and Cystic Components, but is Variable Depending on Content of Fluid
T2* : ± Calcification
MR Angiography : ± A1 Segment of ACA
Differentiation of Cystic CP & RCC“differences that are much more compelling than are their similarities”
Surgery: Tumor Management
ControversialGTR
STR + XRT
STR without adjuvant XRT
~70% Recur/ Progress
Compare
Gross Total Resection
Progression Free Survival
2 88%
Subtotal + XRT
Progression Free Survival
2 91%2 yr 88%
5 yr 67%
Overall Survival
5 yr 98%
10 yr 98%
2 yr 91%
5 yr 69%
Overall Survival
5 yr 99%
10 yr 95%
Isaac Yang, Michael E. Sughrue, Martin J. Rutkowski, Rajwant Kaur, Michael E. Ivan, Derick Aranda, Igor J. Barani, Andrew T. Parsa. Craniopharyngioma: a comparison of tumor control with various treatment strategies. Neurosurgical FOCUS 2010 28:4, E5
Surgery: 3 goals
1.Diagnosis
2.DecompressionHydrocephalusHydrocephalus
EVD v VPS
3.Prevent RecurrenceSurgery TOC
Small Tumors GTR
Surgcial approaches
Bifrontal
Unilateral Subfrontal
Pterional
Transcallosal
Transventricular
Subtemporal
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Surgcial approaches
Transsphenoidal
Intrasellar
Intrasuparsellar infradiaphragmatic
Extended Endoscopic Transphenoidal
Extended Endoscopic Transnasal
Planum Sphenoidale
Olfactory Grove
Clivus
Craniovertebral Junction
Anterior Portion of the Foramen Magnum
Surgical Complications
Endocrine Function
DI, up to 60-93%
Hypogonadism
Panhypopituitarism
Decreased Pituitary Function
Improved Pituitary Function
Permanent DI
Rathke Cleft Cyst 6% 57% 2%
Visual Deterioration
Hypothalamic Injury
Obesity ~40%
Somnolence
Neuropsychologic Disturbance
Cardiovascular Events
Estrogen Deficiency
Craniopharyngioma 31% 0% 39%
Meningioma 9% 0% 0%
Pituitary function after endonasal surgery for nonadenomatous parasellar tumors: Rathke's cleft cysts, craniopharyngiomas, and meningiomas . Joshua R. Dusick, Nasrin Fatemi, Carlos Mattozo, David McArthur, Pejman Cohan, Christina Wang, Ronald S. Swerdloff, Daniel F. Kelly. Surgical Neurology - November 2008 (Vol. 70, Issue 5, Pages 482-490, DOI: 10.1016/j.surneu.2008.03.027)
Nearly All Children With A Nearly All Children With A CraniopharyngiomaCraniopharyngioma Will Have Significant Will Have Significant
Endocrine Problems After SurgeryEndocrine Problems After Surgery
Nearly All Have Anterior And Posterior Nearly All Have Anterior And Posterior Pituitary Deficiency Whether Or Not Pituitary Deficiency Whether Or Not
Complete Tumor Removal Is AchievedComplete Tumor Removal Is Achieved
Most Serious Complication Is Obesity That Most Serious Complication Is Obesity That Develops In About ½ Of Patients Due To Develops In About ½ Of Patients Due To
Hypothalamic Damage (VM)Hypothalamic Damage (VM)
DI Occurs In About 90% Of Postoperative DI Occurs In About 90% Of Postoperative CraniopharyngiomaCraniopharyngioma Patients Patients
Radiotherapy
Adjuvant Treatment
Radiosensitive tumor
Used as adjuvant or with tumor recurrencej
Total dose50 to 65 Gy divided into fractionated doses of 180 to 200 cGy per day
Utility evident for large residual/recurrent tumors and lesions in close proximity to OC
RadiosurgerySRS
Adjunct to surgery, cyst aspiration, & conventional radiation therapy
Doses 9.5 to 16.5 Gy
Fractionate after 13Gy?
Long term studies not available for safety
Control rates for residual or recurrence
70- 92%
Low morbidity
DI, panhypopit, visual loss
<4%
evaluation and effectiveness
Utilized for small tumors < 2cm and > 4 to 5 mm from optic apparatus
Intracavitary Therapy
Instillation of an agent through surgically placed catheter directly into cavity
First used 1952
U d f b th i d dj t th Used for both primary and adjuvant therapy for recurrent cases
RadioisotopesYttrium90, phosphorus32, or rhenium186
Review of literature by Blackburn et al: stereotactic intracavitary therapy
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Chemotherapy
Intracavitary instillation of BleomycinTumor must be Entirely Cystic
IFN-αSquamous Cell Tumors
Survival & Prognostic Factors
Benign histology and rarely disseminateSurvival function of local tumor control
Size of tumor & method of treatment
Tumors > 5cm have much higher recurrence rateTumors > 5cm have much higher recurrence rate
GTR vs. STR + radiotherapy
Biologic factorsAdamantinomous-type higher rate of recurrence after GTR compared to squamous papillary type
Recurrent Craniopharyngiomas
Recur at 2 to 5 years
Previous radical surgery vs. irradiation Reoperaton after radical resection/ radiation is more difficult, making gross total removal less likely, g g y
M&M is also higher
Primary radical surgery
Destroys natural plane between tumor and hypothalamus
Leads to more blunt dissection at reoperation
GTR of recurrent lesion
30 to 68%
Recurrence & Outcome
Treatment for recurrenceEssentially approached in similar fashion as primary tumor
Radiation & surgery offers clear increased relapse-free survival over surgery alone
Recurrent Craniopharyngiomas
Alternative strategy Radiation therapy
Brachytherapy
Initial Rx : STR + radiotherapyRadical resection treatment of choice when feasible plus adjuvant radiation therapy
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Questions?
>50% have a pathognomonicappearance. On these unenhanced and enhanced T1-weighted sagittal images, a compressed pituitary gland can be identified. identified. There is a large intrasellarand suprasellar mass with cystic and enhancing components as well as calcifications. These findings in a child are virtually pathognomonic for craniopharyngioma (perhaps with only a dermoid in the differential diagnosis).
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Differential diagnoses
craniopharyngioma
no gender difference
similar age group
usually suprasellar or have a suprasellar component
t d t l if
Differential diagnoses
Rathke's cleft cysttend to calcify
cystic pituitary adenoma
arachnoid cyst
older patients
no gender difference
epidermoid cyst
usually suprasellar
restriction on DWI
y
pituitary macroadenomawith cystic change
intracranial teratoma
Meningioma
The most common intracranial tumor in adults is the meningioma with 20% of occurring at the skull base. This is an autopsy specimen with the brain This is an autopsy specimen with the brain removed, showing a meningioma sitting on the diaphragma sellae. Meningiomas are almost always solid lesions, sometimes with a cyst on the edge. They can lift up the arachnoid a little bit and enhance uniformly as a general rule.
On the top-left unenhanced and enhanced CT-images, the main differential diagnosis of the enhancing mass would include meningioma, it it d d pituitary adenoma and
an aneurysm. The post-constrast MR-image on the top-right rules out an aneurysm as a possible diagnosis (no flow void), but on axial images a pituitary adenoma and meningioma are still difficult to differentiate.
Notice the spread of the lesion along the meninges.The epicentre of the lesion is above the sella.
On the coronal images (T1 and T1-postcontrast), a compressed pituitary gland can be identified at the bottom of the sella turcica. Above it lies a large mass, partially intrasellar and partially suprasellar.Although the diaphragma sellae can not be identified on these images, it is probably a suprasellar mass growing downwards. When pituitary macroadenomas get this size they usually have areas of hemorrhage or necrosis -in mengiomas this is less often the case.
Hypothalamic and Chiasm Glioma
Gliomas can occur in any part of the brain and the optic chiasm is a common location, particularly in patients with neurofibromatosis type 1. This enhanced CT shows an example of an optic nerve glioma in a patient with neurofibromatosis. There is a suprasellar mass which is indistinguishable from the optic chiasm.
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Postsurgical changes are noted in the sella as well as in the sphenoid sinuses. The major vascular flow-voids are normal. The lesion demonstrates solid and cystic components.
Part of this enhancing mass extends to the region of the optic chiasm.
P ti l ifi ti f th t f th l i i l Th t f th b i d t Partial opacification of the rest of the paranasal sinuses is also seen. The rest of the brain does not show any evidence of ischemic insult. On echo planar diffusion weighted images no unusual signal is seen.
There is suggestion of small amount of air in the suprasellar cistern consistent with recent surgical intervention.
IMPRESSION:
Stable postoperative appearance of the brain following resection and significant debulking of a large sellar and suprasellar mass. Ventricular size is stable and there is no evidence of acute ischemic insult in the brain. Mild paranasal sinusitis is seen.
Schematics illustrating the topographical relationships of the not-strictly-intraventricular type of craniopharyngioma, which corresponds to Type III retroinfundibular in the new classification scheme by Kassamet al. Sagittal (A) and coronal (B) views showing occupation and expansion of the third ventricle floor as caused by the basal part of the lesion. Origin of the lesion from epithelial remnants of the
i h l d t (C) d craniopharyngeal duct (C) and progressive phases of its growth at the third ventricle floor (D–F), following the embryonic theory by Ciric and Cozzens. T = tumor; 1 = ependymal layer; 2 = nervous tissue layer; 3 = piamater; 4 = upper tumoral pole breaking through the ependymal layer; 5 = lower tumoral pole breaking through the pia mater.
Craniopharyngioma
Treatment and prognosis
Treatment is usually surgical with radiotherapy especially useful for incomplete resection. Although benign local recurrence is seen in 7 - 33% of patients
Embryologic Development
Rathke’s pouch
4th week of Embryologic Development
Rostral Outpouching from Roof of Primitive Oral Cavity
Anterior Wall of the Pouch Anterior Lobe of the Pituitary (Pars Distalis)
Posterior Wall of the Pouch DOES NOT Proliferate
I t di t L b f th Pit it (P I t di )Intermediate Lobe of the Pituitary (Pars Intermedia)
Lumen of the Pouch Narrows to form a Cleft
Rathke’s Cleft Normally Regresses
Persistence of this Cleft with Expansion Rathke’s cleft cyst
Cyst wall lined by single columnar cell layer of epithelium, often containing goblet cells, and is often ciliated