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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

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SurgeryManagement of Hydrocephalus

Use of preoperative permanent shunting is debatable

Majority recommend placement of EVD just prior to Majority recommend placement of EVD just prior to craniotomy

If decompression needed

Hydrocephalus often relieved with tumor removal/decompression