NEOPLASMS OF THE MENINGES

James G. Smirniotopoulos, M.D. Professor of Radiology and Neurology

Chairman, Department of Radiology Uniformed Services University of the Health Sciences

4301 Jones Bridge RoadBethesda, MD 20814 USA

Voice: 301-295-3145FAX: 301-295-3893

Email: jsmirnio@usuhs.milVisit us on the WEB at: http://rad.usuhs.mil

DISCLAIMER

The opinions expressed herein are those of the author(s), and are not necessarily representative of the Uniformed Services University of the Health Sciences (USUHS), the Department of Defense (DOD); or the World Health Organization (WHO). Medicine is a constantly changing field, and medical information is subject to frequent correction and revision. Therefore the reader is entirely responsible for verifying the accuracy and relevance of the information contained herein. Portions copyright 1997-2000 James G. Smirniotopoulos, M.D.

Meningioma

• 4 H or 5H Tumor• Most (75%) have Typical Imaging• Atypical Imaging =/= Atypical

Histology• Most (50-75%) Have Edema• Edema ~ Resection ~ Prognosis• Hemangiopericytoma is NOT a

meningioma

Meningioma - 4H/5H

• Hormonally Modulated F >> M• Hemispheric shape• Homogeneous

– hyperdense (CT)– enhancing

• Hyperostosis

PRIMARY MENINGEAL NEOPLASMS

1. MENINGIOMA:

a. Meningioma (typical and metaplastic) b. Atypical Meningioma c. Anaplastic (Malignant) Meningioma2. MESENCHYMAL (non‑meningothelial)3. Primary MELANOCYTIC Lesions4. UNCERTAIN Origin a. Hemangiopericytoma (pericyte) b. Hemangioblastoma (mesenchyme)

MENINGEAL TUMORS: WHO Grades

• MENINGIOMA I• (including most subtypes)• ATYPICAL MENINGIOMA II• PAPILLARY MENINGIOMA III• HEMANGIOPERICYTOMA II III

• ANAPLASTIC MENINGIOMA III

MENINGIOMASCell of Origin

• Dural Fibroblast ?• Arachnoid Cap Cell

– "meningothelial cell"– arachnoid granulations– dural sinuses esp. Sup.

Sag./Sphenoparietal

MENINGIOMABenign Subtypes - WHO I• SYNCYTIAL (Meningothelial)• FIBROBLASTIC (Fibrous)• TRANSITIONAL (Features of Both)• PSAMMOMATOUS• Microcystic (Humid), Secretory• METAPLASTIC FEATURES

– Lipoblastic, Osteoblastic, Chondroblastic

– Myxoid, Xanthomatous, Melanotic

MENINGIOMA:Etiologic Factors

• TRAUMA• RADIATION• VIRUSES• FAMILIAL NON-NF2• NEUROFIBROMATOSIS ‑ Type 2

MENINGIOMARadiation

• Immigrants to Israel (1940's)– Tinea Capitis– Superficial Low Dose (<800

cGray)– High Incidence of Meningioma

• High Dose (> 2000 cGray)

MENINGIOMAMolecular Biology

• Postulate Tumor suppressor Gene• Chromosome 22 ‑ deletion in tumor cells

– both sporadic and inherited– w/ or w/o NF‑2

• Homozygous for TWO wild-type copies is normal

• Heterozygous for 22 develops neoplasm– Because there is a subsequent loss of the

OTHER wild-type gene• Inherited (germline) deletion of 22

– w/Schwannoma = NF2

MENINGIOMAS• 1/7 to 1/4 of all Intracranial Primary

~ 6/ 100k / yearsmall ones in ~ 1.4% of autopsies

• 1/4 ‑ 1/3 of all Intraspinal Tumors• Middle age (40‑60)• Female > Male

cranial 2‑4:1spinal 4‑8:1– Progesterone receptors in 2/3– Estrogen receptors less common

MENINGIOMAS ‑ CT Findings

J. Neurosurg 71:665-672, 1989

• Retrospective, 1980‑1987• 193 Meningiomas, incidence

2.3/100K• Incidence increased to 8.4/100K in

8th decade• 2F:1M Benign, 1:1 for Malignant

Morphology

• Globose (spherical, hemispherical)• en plaque (pancake, crepe, wonton

wrapper, tortilla, pita ...)

MENINGIOMA - Location• Parasagittal 25%• Convexity 20%

• Ant. Basal 40%• Sphenoid 20%• Olfactory 10%• Suprasellar 10%

• Tentorial10%• Ventricular 5%

MENINGIOMAS ‑ CT FindingsJ. Neurosurg 71:665-672, 1989

• LOCATION INCIDENCE• Convexity 34%• Parasagittal 22%• Sphenoid Ridge 17%• Lateral Vent. 5%• Tentorium 4%• CRBLL Convex. 5%• Tuberculum 4%• Olfactory 3%• CPA 2%

MENINGIOMACT Imaging

• Non‑Contrast– Sharply Circumscribed– Homogeneous– Hyperdense (+/‑ Ca++)– NOT from psammoma bodies !– Broad Dural Surface– Bone Changes (Hyperostosis)

• Enhanced CT– Homogeneous Enhancement

MENINGIOMAS: CT Findings (193)

J. Neurosurg 71:665-672, 1989• BENIGN MENINGIOMAS:• Homogeneous Enhancement 72%• Heterogeneous Enhancement 23%• Calcification 27%• Hyperostosis 18%• Midline Shift (large) 72%• "Mushrooming" 0%

MENINGIOMAVasogenic Edema

• VASCULAR– parasitization of MCA, etc.– compression of cortical aa./vv.

• COMPRESSIVE TRAUMA• SECRETORY EFFECT• "TRANSCORTICAL FLOW"

– thinned cortex– gradient from meningioma into

brain

MENINGIOMAS ‑ EDEMANeuroradiology 1986;28:304-312

• Feature w/Edema No Edema• Very Large(>6 cm) 30 9• Large (3-6 cm) 23 11• Small (< 3 cm) 2 1

• HYPER-vascular 41 10• HYPO-vascular 14 11

• Sup Sag Sinus X-ed 3 1• Sphenoparietal X-ed 4 2

MENINGIOMASMR Imaging

• "Meningiomas are ISO‑intense."– usually on T1W– vary pulse sequence to see

• EXTRA‑axial Features– Gray‑matter buckling– Pseudo‑capsule of vessels– Meningeal/dural "TAIL"

• GADOLINIUM ENHANCEMENT

MENINGIOMA*Imaging Features: CT vs. MR

• Mass effect 88% 90%• Extraaxial Location 42% 70%• Broad Dural Attach. 74% 98%• Typical. Dense./Intensity 92% 74%• Hyperostosis 10% 14%• Homogeneous 76% 76%• Enhancement 96%(78%) 96%(80%)

(Homogeneous) • Meningeal. Enhance 2% 50%• “Capsule” 14% 68%

• *Neuroradiology 1990;32: 467-473

MENINGIOMAAngiography - Supply

• External Carotid 85%• Internal Carotid 63%• Tumor Blush 95%

MENINGIOMAAngiography - Transit

Time• Blush or Stain

– early arterial– prominent in VENOUS phase– capillaries/sm. arterioles– (too small to see individually)

• Venous Filling– characteristic if delayed– may fill with/ before Nl. vv.

MENINGIOMAAngiography - Transit

Time• Venous Filling (Stattin,1966)

– 170 Meningiomas– delayed in 136 (80%)– with Nl. vv. in 10 (6%)– earlier in 24 (14%) – ( 8 in early arterial phase)

• Leeds & Taveras (1969)– EDV in 6/36 (16%)

MENINGIOMAAngiography - Tumor

Blush• May come early• Usually very DENSE• Stays way too long• (Persistent!)

• The “In-Law Effect”

MENINGIOMAEffect on Skull

• Hyperostosis– w or w/o micro invasion

• Pressure Erosion– periosteal remodeling

• Bone Destruction– (micro invasion)

HYPEROSTOSIS IN MENINGIOMAS

MENINGIOMAHyperostosis

• 15 - 90% of Meningioma– more common with subfrontal and

en plaque• Does NOT prove invasion of bone

– “sympathetic change” (Alk. Phos.?)• Implies CHRONICITY and benign

behavior• Skull Base

– Invasion via Haversian Canals

Hyperostosis - DDX

• Increased Density– Paget Disease– Fibrous Dysplasia– Metastasis

• Breast• Prostate• Lymphoma

– Ca++ Hematoma• SDH• Cephalohematom

a

• Widened Diploe– Paget disease– Fibrous Dysplasia– Dilantin Tx– Tx Hydrocephalus– Ca++ Hematoma

• SDH• Cephalohematoma

Atypical “Meningioma”

• Atypical Imaging– 25% of meningioma

• Atypical Pathology– Atypical meningioma ( 5- 7%)– Malignant meningioma ( 1-3%)

• Hemangiopericytoma ( 1.5 - 2.5 %)

MENINGIOMAAtypical Imaging

• Focal Lucency Outside (arachnoid cyst)

• Focal Lucency Inside (necrosis, cyst)• Hypodensity ("humid", lipoblastic)• Focal Hyperdensity (metaplasia,

hemorrhage)• Heterogeneous• Hyperintensity on T1W• Hyperintensity on T2W

MENINGIOMACysts

• Inside of neoplasm– (rim enhancement)

• Between tumor and brain– ("arachnoid cysts")

• Inside Brain– PIA separates tumor from brain– ?? Result of Chronic Edema– Vacuolization of White Matter

MENINGIOMAAtypical Histology

• Atypical Meningioma ~ 5-7 % • Anaplastic Meningioma ~ 1-3%

~ 0.2 / 100k per year• Higher incidence of Recurrence• Shorter time to Recurrence• "Atypical Histology"

necrosisexcessive mitoses* invasion into brain

Meningioma:Brain Invasion

• Independent Variable for Prognosis– Decreases Resectability– Increases Recurrence

• Seen more often with Atypical/Anaplastic– Can occur with “benign”

meningioma• Associated with EDEMA

– but not necessary for Edema

MENINGIOMA“Malignant

Meningioma”• Anaplastic (Malignant) Meningioma• Papillary Meningioma• "Benign" Metastasizing

Meningioma• Hemangio‑Peri‑Cytoma (HPC)• Malignant Fibrous Histiocytoma

(MFH)

Hemangiopericytoma

• Syn: “angioblastic meningioma”• Cell of Origin - perivascular

pericyte of Zahn• <1% of primary CNS• M 1.4:1 F• Age - 40’s• Dural based, bone destruction,

lobulated

HEMANGIOPERICYTOMA(HPC)

• Narrow dural base ("Mushrooming")• No Hyperostosis• Lobulated (not hemispheric)• Internal Signal Voids (on MRI)

– irregular and multiple• Hypervascular on Angio

– irregular patterns

MENINGIOMARadiologic Features -

CT• Feature Benign/Malignant• Homogeneous Enhancement 72% /36%

• Heterogeneous Enhancement 23% /64%

• Hyperostosis 18% / 7%• Calcification 27% / 0%• “Mushrooming” 0% /57%

– narrow attachment and larger “cap” invaginating into brain

HEMANGIOPERICYTOMAParkway Deli Sign

• Parkway Delight Reuben• PastramiCorned Beef• Cole Slaw Sauerkraut• Muenster Swiss Cheese• Thousand Island Russian Dressing

• PumpernickelRye Bread

Parkway Delight Vs. Rueben

Pastrami Corned BeefColeslaw Sauerkraut

Thousand lsand Russian

Muenster SwissPumpernikel Rye Bread

Just Like a Rueben!Except ...

Parkway Delight Vs. Rueben

Pastrami Corned BeefColeslaw Sauerkraut

Thousand lsand Russian

Muenster SwissPumpernikel Rye Bread

HEMANGIOPERICYTOMAParkway Deli Sign

• Hemangiopericytoma <> Meningioma

• Narrow Base Broad-based• Bone Destruction Hyperostosis• Heterogeneous Homogeneous• Large Irregular Vessels Spoke-

wheel• Lobulated Hemispheric

Hemangiopericytoma Vs. Meningioma

Narrow Base Broad BaseLobulated Hemisphere

Heterogeneous Homogeneous

Bone Destruction HyperostosisLarge I rregular

VesselsSpoke Wheel

Vessels

MENINGIOMA Summary: The 4H - 5H

Tumor• Benign, slowly growing• Arachnoid Cap Cell, meningothelial• Sharply Circumscribed EXTRA‑axial• Hemispheric / en plaque• Broad‑based on DURA• HPC is NOT a meningioma

Meningioma:Suggestive Features

• Homogeneous Hemispheric Mass• Broad Dural Base• Hyperostosis• Transdural Mass (falx, tentorium)• Hyperdense on Plain CT• Dural Tail • ~ GM Signal on MR

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