eMedicine Specialties > Neurology > Neuro-oncology
Meningioma: Differential Diagnoses & Workup
Updated: Jun 30, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Differential Diagnoses
Other Problems to Be Considered
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Workup
Laboratory Studies
No specific laboratory tests are used to screen for meningioma.
Imaging Studies
- Imaging studies are the mainstay of diagnosis (see Media files 1-7).
Case 1: Surgical view of the tumor. The dura is opened, and the meningioma can be seen extending en plaque over the surface of the brain.
- Plain skull radiograph may reveal hyperostosis and increased vascular markings of the skull, as well as intracranial calcifications.
- On plain head CT scans, meningiomas are usually dural-based tumors that are isoattenuating to slightly hyperattenuating.
- They enhance homogeneously and intensely after the injection of iodinated contrast material.
- Perilesional edema may be extensive. Hyperostosis and intratumoral calcifications may be present.
- The tumor compresses the brain without invading it.
- Multiple meningiomas may be difficult to differentiate from metastasis.
- On T1- and T2-weighted MRIs, the tumors have variable signal intensity. If a meningioma is suspected, obtaining an enhanced MRI is imperative.
- Meningiomas enhance intensely and homogeneously after injection of gadolinium gadopentetate.
- The edema may be more apparent on MRI than on CT scanning.
- An enhancing tail involving the dura may be apparent on MRI.
- Cystic meningiomas may exhibit intratumoral or peritumoral cysts. The peritumoral cysts may actually represent a gliotic response and may not necessitate surgical extirpation.
- Endovascular angiography allows the surgeon to preoperatively determine the vascularization of the tumor and its encroachment on vital vascular structures.
- Late venous images are important to determine the patency of the involved dural sinuses.
- Angiographic features of meningiomas include the following:
- Supply from the external circulation
- Mother-in-law blush (which comes early and leaves late)
- Sunburst or radial appearance of the feeding arteries
- Although magnetic resonance arteriography (MRA) and magnetic resonance venography (MRV) have decreased the role of classical angiography, the latter remains a powerful tool for planning surgery.
- Angiography is still indispensable if embolization of the tumor is deemed necessary.
- New research tools such as positron emission tomography (PET), including octreotide-PET, or magnetic resonance spectroscopy (MRS) have been used to predict in vivo the aggressiveness of meningiomas.10
Procedures
- Preoperative endovascular embolization of the vascular feeders from the external circulation may be beneficial in extremely vascular meningiomas.11
- If this is the case, resection should be performed shortly after embolization to decrease the likelihood of tumor revascularization.
Histologic Findings
Meningiomas are usually globular, well-demarcated neoplasms. They have a wide dural attachment and become invaginated into the underlying brain without invading it. Their cut surface is either translucent pale or homogeneously reddish brown. It may be gritty on cutting. Some meningiomas occur as a sheetlike extension that covers the dura but does not invaginate the parenchyma; this variant is called meningioma en plaque. The last morphologic variant is the cavernous sinus meningioma that infiltrates the cavernous sinus and becomes interdigitated with its contents. The 3 most common histologic subtypes of meningiomas are the meningothelial (syncytial), transitional, and fibroblastic meningiomas. See Media files 8-9 for representative pathologic views of various subtypes.
Case 2: Bone flap was removed. Note tumoral breach of the dura. The dura and overlying skull were removed surgically. Duraplasty and cranioplasty were performed
Case 2: Surgical specimen. Complete resection was achieved.
Meningothelial meningiomas reveal densely packed cells that are arranged in sheets with no clearly discernible cytoplasmic borders. Although not prominent, whorls are present (calcified whorls are termed psammoma bodies). Nuclei show intranuclear vacuoles.
Fibroblastic (fibrous) meningiomas reveal sheets of interlacing spindle cells. The intercellular stroma is composed of reticulin and collagen. The transitional variety reveals features common to both the meningothelial and fibroblastic varieties; others include angiomatous, microcystic, secretory, clear cell, choroid, lymphoplasmacyte-rich, papillary, and metaplastic variants.
Meningiomas may be associated with hyperostosis.2 The exact nature of the cause of this hyperostosis is controversial (ie, reactive versus tumoral infiltration).
Immunohistochemistry
Immunohistochemistry can help diagnose meningiomas, which are positive for epithelial membrane antigen (EMA) in 80% of cases. They stain negative for anti-Leu 7 antibodies (positive in schwannomas) and for glial fibrillary acidic protein (GFAP). Progesterone receptors can be demonstrated in the cytosol of meningiomas; the presence of other sex hormone receptors is much less consistent. Somatostatin receptors also have been demonstrated consistently in meningiomas.
Malignancy
The notion of malignancy in meningiomas is still vague.12 Some histologic variants, such as papillary meningioma, undoubtedly carry a less favorable prognosis than other histologic types. Two features are considered clear signs of malignancy: cortical invasion by the tumor and distal metastasis. Of note, in the 2007 WHO grading scheme, brain invasion is considered a criterion for atypia.
Several stains have been used to help predict the behavior of meningiomas. These stains quantify the mitotic rate of these tumors. Bromodeoxyuridine (BudR) labeling requires an intravenous (IV) injection before tumor removal. On the other hand, immunohistologic staining for proliferating cell nuclear antigen (PCNA) can be performed on fixed specimens. Some have attempted to correlate the pathology and behavior of meningiomas to the loss of specific genetic material.
The World Health Organization classification of meningiomas is presented in Table 2.
Table 2. Summary of the 2007 WHO Grading Scheme for Meningiomas
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Table
| WHO Grade | Histological Subtype | Histological Features |
| I | Meningothelial, fibroblastic, transitional, angiomatous, microcystic, secretory, lymphoplasmacytic metaplastic, psammomatous | Does not fulfill criteria for grade II or III |
| II (Atypical) | Chordoid, clear cell | 4 or more mitotic cells per 10 hpf and/or 3 or more of the following: increased cellularity, small cells, necrosis, prominent nucleoli, sheeting, and/or brain invasion in an otherwise Grade I tumor |
| III (Anaplastic) | Papillary, rhabdoid 13 | 20 or more mitoses per 10 hpf and/or obviously malignant cytological characteristics such that tumor cell resembles carcinoma, sarcoma, or melanoma |
| WHO Grade | Histological Subtype | Histological Features |
| I | Meningothelial, fibroblastic, transitional, angiomatous, microcystic, secretory, lymphoplasmacytic metaplastic, psammomatous | Does not fulfill criteria for grade II or III |
| II (Atypical) | Chordoid, clear cell | 4 or more mitotic cells per 10 hpf and/or 3 or more of the following: increased cellularity, small cells, necrosis, prominent nucleoli, sheeting, and/or brain invasion in an otherwise Grade I tumor |
| III (Anaplastic) | Papillary, rhabdoid 13 | 20 or more mitoses per 10 hpf and/or obviously malignant cytological characteristics such that tumor cell resembles carcinoma, sarcoma, or melanoma |
More on Meningioma |
| Overview: Meningioma |
 Differential Diagnoses & Workup: Meningioma |
| Treatment & Medication: Meningioma |
| Follow-up: Meningioma |
| Multimedia: Meningioma |
| References |
| Further Reading |
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References
Evans DG. Neurofibromatosis type 2: genetic and clinical features. Ear Nose Throat J. Feb 1999;78(2):97-100. [Medline].
Pieper DR, Al-Mefty O, Hanada Y, Buechner D. Hyperostosis associated with meningioma of the cranial base: secondary changes or tumor invasion. Neurosurgery. Apr 1999;44(4):742-6; discussion 746-7. [Medline].
Majchrzak K, Tymowski M. Surgical treatment of the tentorial and falco-tentorial junction meningiomas. Minim Invasive Neurosurg. Apr 2009;52(2):93-7. [Medline].
Arima T, Natsume A, Hatano H, Nakahara N, Fujita M, Ishii D, et al. Intraventricular chordoid meningioma presenting with Castleman disease due to overproduction of interleukin-6. Case report. J Neurosurg. Apr 2005;102(4):733-7. [Medline].
Ragel BT, Jensen RL, Couldwell WT. Inflammatory response and meningioma tumorigenesis and the effect of cyclooxygenase-2 inhibitors. Neurosurg Focus. 2007;23(4):E7. [Medline].
Kim JH, Lee SH, Rhee CH, et al. Loss of heterozygosity on chromosome 22q and 17p correlates with aggressiveness of meningiomas. J Neurooncol. Nov 1998;40(2):101-6. [Medline].
Albrecht S, Goodman JC, Rajagopolan S, Levy M, Cech DA, Cooley LD. Malignant meningioma in Gorlin's syndrome: cytogenetic and p53 gene analysis. Case report. J Neurosurg. Sep 1994;81(3):466-71. [Medline].
Verstegen MJ, van den Munckhof P, Troost D, Bouma GJ. Multiple meningiomas in a patient with Rubinstein-Taybi syndrome. Case report. J Neurosurg. Jan 2005;102(1):167-8. [Medline].
Milham S. Meningioma and mobile phone use. Int J Epidemiol. Apr 22 2009;[Medline].
Lee JW, Kang KW, Park SH, Lee SM, Paeng JC, Chung JK, et al. (18)F-FDG PET in the assessment of tumor grade and prediction of tumor recurrence in intracranial meningioma. Eur J Nucl Med Mol Imaging. Apr 18 2009;[Medline].
Kerim AA, Bonneville F, Jean B, Cornu P, Lejean L, Chiras J. Balloon-assisted embolization of skull base meningioma with liquid embolic agent. J Neurosurg. Apr 10 2009;[Medline].
Rosenberg LA, Prayson RA, Lee J, Reddy C, Chao ST, Barnett GH, et al. Long-term experience with World Health Organization grade III (malignant) meningiomas at a single institution. Int J Radiat Oncol Biol Phys. Jun 1 2009;74(2):427-32. [Medline].
Dutta D, Lee HN, Munshi A, Gupta T, Kane S, Sridhar E, et al. Intracerebral cystic rhabdoid meningioma. J Clin Neurosci. May 7 2009;[Medline].
Norden AD, Drappatz J, Wen PY. Advances in meningioma therapy. Curr Neurol Neurosci Rep. May 2009;9(3):231-40. [Medline].
Chamberlain MC, Tsao-Wei DD, Groshen S. Temozolomide for treatment-resistant recurrent meningioma. Neurology. Apr 13 2004;62(7):1210-2. [Medline].
Milker-Zabel S, Huber P, Schlegel W, Debus J, Zabel-du Bois A. Fractionated stereotactic radiation therapy in the management of primary optic nerve sheath meningiomas. J Neurooncol. Apr 1 2009;[Medline].
Smith JL, Vuksanovic MM, Yates BM, Bienfang DC. Radiation therapy for primary optic nerve meninigiomas. J Clin Neuroph. 1981;1:85-99. [Medline].
Mirimanoff RO. New radiotherapy technologies for meningiomas: 3D conformal radiotherapy? Radiosurgery? Stereotactic radiotherapy? Intensity-modulated radiotherapy? Proton beam radiotherapy? Spot scanning proton radiation therapy. . . or nothing at all?. Radiother Oncol. Jun 2004;71(3):247-9. [Medline].
Nutting C, Brada M, Brazil L, et al. Radiotherapy in the treatment of benign meningioma of the skull base. J Neurosurg. May 1999;90(5):823-7. [Medline].
Kondziolka D, Levy EI, Niranjan A, et al. Long-term outcomes after meningioma radiosurgery: physician and patient perspectives. J Neurosurg. Jul 1999;91(1):44-50. [Medline].
Kondziolka D, Niranjan A, Lunsford LD, Flickinger JC. Stereotactic radiosurgery for meningiomas. Neurosurg Clin N Am. Apr 1999;10(2):317-25. [Medline].
Strassner C, Buhl R, Mehdorn HM. Recurrence of intracranial meningiomas: did better methods of diagnosis and surgical treatment change the outcome in the last 30 years?. Neurol Res. Jun 2009;31(5):478-82. [Medline].
Al-Mefty O, Smith R. Clival and petroclival meningiomas. In: Al-Mefty O, ed. Meningiomas. New York, NY: Raven; 1991.
Alexiou GA, Vartholomatos G, Tsiouris S, Papadopoulos A, Kyritsis AP, Polyzoidis KS, et al. Evaluation of meningioma aggressiveness by (99m)Tc-Tetrofosmin SPECT. Clin Neurol Neurosurg. May 7 2008;[Medline].
Black P, Kathiresan S, Chung W. Meningioma surgery in the elderly: a case-control study assessing morbidity and mortality. Acta Neurochir (Wien). 1998;140(10):1013-6; discussion 1016-7. [Medline].
Cappabianca P, Cirillo S, Alfieri A, et al. Pituitary macroadenoma and diaphragma sellae meningioma: differential diagnosis on MRI. Neuroradiology. Jan 1999;41(1):22-6. [Medline].
De Monte F, Al-Mefty O. Meningiomas. In: Kaye AH, Laws ER, eds. Brain Tumors: An Encyclopedic Approach. Ediburgh, Scotland: Churchill Livingstone; 1995: 675-704.
Drummond KJ, Zhu JJ, Black PM. Meningiomas: updating basic science, management, and outcome. Neurologist. May 2004;10(3):113-30. [Medline].
Feldman RP, Marcovici A, Suarez M, Goodrich JT. Foreign body granuloma mimicking intracranial meningioma: case report and review of the literature. Neurosurgery. Apr 1999;44(4):855-8. [Medline].
Haddad GF, Al-Mefty O. Approaches to petroclival tumors. In: Wilkins RH, Rengachary SS, eds. Neurosurgery. Vol 2. 2nd ed. New York, NY: McGraw-Hill; 1996:1695-706.
Haddad GF, Al-Mefty O. Meningiomas: an overview. In: Wilkins RH, Rengachary SS, eds. Neurosurgery. Vol 1. 2nd ed. New York, NY: McGraw-Hill; 1996:833-42.
Haddad GF, Al-Mefty O. The road less traveled: transtemporal access to the CPA. Clinical Neurosurgery. 1994;41:150-167.
Iwai Y, Yamanaka K, Yasui T, et al. Gamma knife surgery for skull base meningiomas. The effectiveness of low-dose treatment. Surg Neurol. Jul 1999;52(1):40-4; discussion 44-5. [Medline].
Jaffrain-Rea ML, Minniti G, Santoro A, et al. Visual improvement during octreotide therapy in a case of episellar meningioma. Clin Neurol Neurosurg. Mar 1998;100(1):40-3. [Medline].
Kleihues P, Cavanee W. World Health Organization Classification of Tumours: Pathology and Genetics: Tumours of the Nervous System. Lyon, France: IARC; 2000.
Klutmann S, Bohuslavizki KH, Tietje N, et al. Clinical value of 24-hour delayed imaging in somatostatin receptor scintigraphy for meningioma. J Nucl Med. Aug 1999;40(8):1246-51. [Medline].
Kotzen RM, Swanson RM, Milhorat TH, Boockvar JA. Post-traumatic meningioma: case report and historical perspective. J Neurol Neurosurg Psychiatry. Jun 1999;66(6):796, 798. [Medline].
Larner AJ, Ball JA, Howard RS. Sarcoid tumour: continuing diagnostic problems in the MRI era. J Neurol Neurosurg Psychiatry. Apr 1999;66(4):510-2. [Medline].
Lee GK, Coel M, Ko J, Tom B. Two meningiomas detected incidentally by Tc-99m HDP bone scintigraphy during a work-up for breast cancer. Clin Nucl Med. Jul 1999;24(7):525-6. [Medline].
Liu M, Liu Y, Li X, Zhu S, Wu C. Cystic meninigioma. J Clin Neurosci. Sep 2007;14(9):856-9. [Medline].
Lusis E, Gutmann DH. Meningioma: an update. Curr Opin Neurol. Dec 2004;17(6):687-92. [Medline].
Nakasu S, Nakasu Y, Nakajima M, et al. Preoperative identification of meningiomas that are highly likely to recur. J Neurosurg. Mar 1999;90(3):455-62. [Medline].
Norden AD, Drappatz J, Wen PY. Targeted drug therapy for meningiomas. Neurosurg Focus. 2007;23(4):E12. [Medline].
Perry A, et al. Meningiomas. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, Jouvet A, et al. World Health Organization Classification of Tumours of the Central Nervous System. 4th ed. Lyon: IARC; 164-172.
Rempel SA, Ge S, Gutierrez JA. SPARC: a potential diagnostic marker of invasive meningiomas. Clin Cancer Res. Feb 1999;5(2):237-41. [Medline].
Runzi MW, Jaspers C, Windeck R. Successful treatment of meningioma with octreotide [letter]. Lancet. May 13 1989;1(8646):1074. [Medline].
Sharif S, Brennan P, Rawluk D. Non-surgical treatment of meningioma: a case report and review. Br J Neurosurg. Aug 1998;12(4):369-72. [Medline].
Vaicys C, Schulder M, Wolansky LJ, Fromowitz FB. Falcotentorial plasmacytoma: case report. J Neurosurg. Jul 1999;91(1):132-5. [Medline].
Whittle IR, Smith C, Navoo P, Collie D. Meningiomas. Lancet. May 8 2004;363(9420):1535-43. [Medline].
Further Reading
Clinical guidelines
Improving outcomes for people with brain and other CNS tumours.
National Collaborating Centre for Cancer - National Government Agency [Non-U.S.]. 2006 Jun. 180 pages. NGC:005147
Gu ideline for the diagnosis, investigation and management of polycythaemia/erythrocytosis.
British Committee for Standards in Haematology - Professional Association. 2005 Jul. 22 pages. NGC:006179
ACR Appropriateness Criteria® orbits, vision, and visual loss.
American College of Radiology - Medical Specialty Society. 1999 (revised 2006). 9 pages. NGC:005122
Clinical trials
Phase II Trial of Sunitinib (SU011248) in Patients With Recurrent or Inoperable Meningioma
Monthly SOM230C for Recurrent or Progressive Meningioma
Biobank Meningioma: Storing Blood for Analysis of DNA and Protein of Patients With Meningioma
Related eMedicine topics
Meningioma, Brain
Meningioma, Spine
Meningioma, Optic Nerve Sheath
Meningioma, Sphenoid Wing
Skull Base Tumors
Keywords
meninges, meningeal carcinoma, meningeal cancer, arachnoidal cap cells, primary intracranial neoplasms, asymptomatic meningioma, neurofibromatosis-2, NF-2, familial meningiomas, primary intracranial tumors, hyperostosis, seizures, dysphasia, disinhibited behavior, somnolence, urinary incontinence, anosmia, ipsilateral optic atrophy
contralateral papilledema, Kennedy-Foster syndrome, diplopia, facial numbness, contralateral hemianopsia, facial weakness, Brown-Sequard syndrome, hemispinal cord syndrome, exophthalmos, monocular loss of vision, blindness, ipsilateraldilated pupil, monocular optic nerve swelling, optociliary shunt vessels, multiple cranial nerve palsies, paraparesis, sphincteric troubles, tongue atrophy
transient ischemic attack–like episodes, TIA–like episodes, stroke, intraventricular meningiomas, obstructive hydrocephalus, panhypopituitarism, visual field defects, raised intracranial pressure, brain herniation, decreased mentation, decreased facial sensation, facialparesis, decreasedhearing, deviation of uvula, hemiatrophy of tongue, pronator drift,hyperreflexia, positive Hoffman sign, Babinski sign, parietal-lobe syndrome, Gerstmann syndrome, agraphia, acalculia, right-left disorientation, finger agnosia, tactile extinction, neglect of contralateral side
visual extinction, congruent homonymous hemianopsia, spinal meningiomas, decreased pain sensation, quadriparesis, sphincteric weakness, ipsilateral weakness, decrease in position sense, cranial irradiation, chromosome 22q, merlin, schwannomin, anaplastic meningioma, monosomy of chromosome 7, loss of progesterone receptors, increased expression of ornithinedecarboxylase, increased expression of cyclooxygenase 2, radiation-induced meningiomas, matrix metalloproteinases, MMPs, tissue inhibitors of MMPs, TIMPs
