B Pavani et al. Angiomatous Meningioma.
ANGIOMATOUS MENINGIOMA - DIAGNOSTIC EVALUATION WITH LITERATURE REVIEW
Pavani B.1, Indrasen Reddy K.2,Vishwaksena Reddy P.3, Sanjeev Choudri4
1Associate Professor, Department of Pathology, Malla Reddy Medical College for Women, Hyderabad 2Consultant General Physician, 3Consultant neurosurgeon, 4Consultant Radiologist: Ozone hospitals Kothapet Hyderabad, Telangana.
Corresponding author: Dr .V. Pavani, Associate Professor, Department of Pathology, Malla Reddy Medical College for Women, Hyderabad.
This article may be cited as: B Pavani, K R Indrasen, P R Vishwaksena, Choudri S. Angiomatous Meningioma - Diagnostic evaluation with literature review. J Adv Med Dent Scie Res 2016;4(1):114-118.
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B Pavani et al. Angiomatous Meningioma.
CASE REPORT:
A 60 year old male patient presented with complaint of fainting spells for one year right hemiparesis of sudden onset. His general examination was otherwise normal. All haematological and serological tests were within normal limits. Magnetic Resonance Imaging (MRI) of brain showed homogenously enhancing mass lesion located at left falx cerebri (Fig-1). Patient underwent craniotomy with total excision of the tumor. Grossly the tumor was sent in a piecemeal of greyish brown soft tissue bits, altogether amounting to 5ml.Tissue was formalin fixed and paraffin embedded. Five-micrometer-thick sections were prepared for hematoxylin and eosin (H&E) staining. Microscopic examination showed a predominantly vascular tumour with only rare presence of benign nests of meningothelial cells. Individual cells had delicate round to oval nuclei and eosinophilic cytoplasm. There were abundant small capillary like thin walled blood vessels accounting to more than 50% of the
overall lesion, along with areas of hyalinization of vessel wall , luminal thrombosis and focal hemorrhages. No evidence of any atypia or malignancy seen (Fig 2, 3, 4 and 5).
DISCUSSION
Meningiomasare a diverse set of tumors arising from themeninges. These tumors usually arebenignin nature; however, a small percentage aremalignant. Meningiomas present rarely in children, which show extraaxial location.1 Many meningiomas areasymptomatic, producing no symptoms throughout the life time. The term "meningioma" was first used byHarvey Cushing(1869–1939) in 1922, to describe a set of tumors that occur throughout theneuraxis(brain and spinal cord).Charles Oberling then separated these into subtypes based on cell structure, and over the years, several other researchers have defined dozens of different subtypes as well.2
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Figure 1: Falcine meningioma: extraparenchymatous dense mass with broad base implantation on left parafalcine region , homogenously enhancing on post contrast T1 weighted images.
Figure 2 Figure 4
Figure 3 Figure 5
Figure 2&3: Angiomatous meningioma (Hematoxylin and Eosin stain (40&10X). Photomicrograph with clusters of benign meningothelial cells. Figure-4: Angiomatous meningioma (Hematoxylin and Eosin stain 10X). Photomicrograph depicting numerous vessels accounting for more than 50% of the lesion. Figure 5: Angiomatous meningioma (Hematoxylin and Eosin stain 40X). Photomicrograph showing rich thick-walled blood vessels often with very fibrous&hyalinized walls.
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Since meningiomas are extra-axial and vascularized, they are visualized promptly with arcontrastCT,MRIwithgadolinium, and arteriography. Meningiomas show isointensity or hyperintensity to the cerebral cortex under magnetic resonance imaging (MRI) producing a short extension of contrast enhancing tissue along the dura (dura tail),the latter considered to be a valuable diagnostic feature(9,3). Radiographically, there is no specific feature to help in the subclassification of meningiomas. Perilesional edema is usually observed in WHO Grade II meningiomas,with the exception of angiomatous meningiomas which belongs to WHO grade I.3,4Tamiyaet alexamined the radiological and histological features influencing the development of peritumoral brain edema and analyzed 125 patients with primary intracranial meningiomas.
Many hypotheses were put forward to explain the pathogenesis of peritumoral edema in a meningioma, such as the role of vascular endothelial growth factor and pial blood supply. Multiple contributing factors have been proposed including tumor size, histology, intratumoral venous congestion, and ischemia to adjacent brain due to tumor compression. Of these, the overexpression and tumoral secretion of vascular endothelial growth factor (VEGF) has been proved to be associated with the extent of peritumoral edema. VEGF is known to be a potent inducer of capillary permeability and excessive cerebral and pial blood supply. It is expressed by both low and high grade meningiomas leading to peritumoral edema.5,6
In 1979, theWorld Health Organization(WHO) classified seven subtypes, upgraded in 2000 to a classification system with nine low-grade variants (grade I tumors) and three variants each of grade II and grade III meningiomas.2The WHO classification of 2000 edition aims for precise prediction of the divergent clinical characteristics of meningiomas with a histological grading system based on statistically significant clinicopathological correlations. The upgraded current edition of WHO classification 2007 is the most utilized version,which is based on the consensus forwarded by an international Working Group of 25 pathologists ,geneticists and contributions from upto 70 international experts.2,7
The only change between the WHO 2007 and 2000 edition is that brain-infiltrative and otherwise benign meningiomas are classified as grade II.
WHO grades meningiomas on a scale from I to III .
The majority of meningiomas are WHO Grade I, and ar are graded by subtypes.6,7
§ Benign (Grade I) – (90%) – meningothelial, fibrous, transitional, psammomatous, Angiomatous (includes hemangioblastic, angioblastic), Microcystic ,Secretory, Metaplastic, Lymphoplasmacyte-rich.
§ Atypical (Grade II) – (8%) – chordoid, clear cell or atypical by criteria
§ Anaplastic/malignant (Grade III) – (2%) – Rhabdoid and papillary subtypes, or anaplastic by criteria (most aggressive)
Metastasis is relatively rare in meningioma which constitutes only 0.1% of cases .However they can spread to other parts of thebrainand often to thelungs.8
Angiomatous meningioma is an uncommon subtype having a predilection for the cerebral convexity.It has also been reported from other rare sites,such as tonsil. Angiomatous meningioma demonstrates some distinct features compared to other benign meningiomas.Hasselblatt,Martin etal studied fairly large number,accounting to 38 cases of angiomatous meningiomas and therefore concluded that an angiomatous meningioma displays some unique differentiating features ,which is indeed a justified sub group among meningiomas.9,10 Martin et al (2004) proposed that the male to female ratio for an angiomatous meningioma is much higher than that for a conventional meningioma. Early stereotactic biopsy is needed to establish the diagnosis and is often recommended.11 On histopathology, angiomatous meningioma is chararcterised by abundance of well-formed vascular channels, sinusoids or capillaries exceeding 50% of the tumor area. At least focal meningothelial differentiation would be noted in all tumors especially in the hypercellular areas. Mitotic activity is usually sparse. Since angiomatous meningiomas are characterized by high vascularity, differential diagnosis mainly includes vascular tumors such as hemangiopericytoma and capillary hemangioblastoma as detailed by Rao et al.12,13) An angiomatous meningioma at least focally exhibits classic meningothelial morphology,in contrast to the vascular neoplasms 13 forms the key differentiating feature. Studies by Martin et al showed two subtypes of angiomatous meningioma, viz., microvascular subtype (more than 50% contain vessels with diameter below 30 μ), and a macrovascular subtype(with viceversa features).14 Xanthomatous degeneration, moderate nuclear pleomorphism and metaplasia may be seen but have no prognostic significance.Xanthomatous degeneration is attributed to leakage of plasma lipids across thin vessel walls, as was the feature seen in our case.
Microscopic grading is based on increased cellularity, high nucleocytoplasmic ratio, large prominent nucleoli, patternless sheets, significant mitosis, and spontaneous or geographic necrosis. Hence, a complete workup includes the assessment of grade. Invasion of dura, bone or soft tissues and presence of pleomorphic or atypical nuclei do not affect the grading.
Immunohistochemistry holds particular diagnostic value in cases of anaplastic meningioma, although it needs to be emphasised that even immunohistochemical evidence of meningothelial differentiation can be lacking in some patients. An overall thorough histopathological analysis with a search for meningotheliomatous differentiation, complimented by a minimum of two confirmatory immunohistochemistry markers, namely EMA &KI67 are of immense help in the final diagnosis. An objective method of evaluating the proliferative activity is by performing Ki-67/MIB-1 immunostaining on tissue sections.
In our case the mean MIB-1(Ki-67) proliferation index was 1.7 % &no brain invasion was identified15. In light of all the above substantiating findings, the tumor was finally labelled as angiomatous haemangioma,WHO grade I.
Angiomatous meningiomas do not demonstrate common mutations found in other variants of meningioma. A study by Malak S. Abedalthagafi etal demonstrates that angiomatous meningiomas have distinct genomic features that may be clinically useful for their diagnosis.16
CONCLUSION
To summarize ,an angiomatous meningioma may pose diagnostic difficulty to the pathologists.An intense search for benign meningotheliomatous differentiation via the assistance of immunohistochemistry markers mainly by EMA and KI67, is of immense help. Since identification of angiomatous meningioma may have prognostic implications,it is therefore important that the existence of this rare subgroup of highly vascularized meningioma is recognized.
REFERENCES:
1. Drake JM, Hendrick EB, Becker LE. Chuang SH. Hoffman HJ , Humphreys RP. Intrac ranial meningiomas in children. Pediatr f'leurosci 1985: 12: 134-1 39
2. Park, Bong Jin; Kim, Han Kyu; Sade, Burak; Lee, Joung H. "Epidemiology", Meningiomas, pp. 11–13.
3. Kim BW, Kim MS, Kim SW, Chang CH, Kim OL. Peritumoral brain edema in meningiomas: correlation of radiologic and pathologic features. J Korean Neurosurg Soc. 2011; 49:26–30.
4. Tamiya, T., Ono, Y., Matsumoto, K., Ohmoto, T. 2001. Peritumoral brain edema in intracranial meningiomas: Effects of radiological and histological factors. Neurosurgery, 49: 1046–1051.
5. Department of Neurosurgery, Copenhagen University HospitalIntracranial meningiomas, the VEGF-A pathway, and peritumoral brain edema. Danish Medical Journal 04/2013; 60(4):B4626.
6. Nassehi D,Sørensen LP,Dyrbye H,Thomsen C,Juhler M,Laursen H,Broholm HJ Neurosurg.Peritumoral brain edema in angiomatous supratentorial meningiomas: an investigation of the vascular endothelial growth factor A pathway.2003 Oct; 99(4):787-91.
7. Perry A, Louis DN, Scheithauer BW, Budka H, Von Deimling A. Meningiomas. In: Lous DN, Oghaki H, Wiestler OD, Cavenee WK, editors WHO Classification of Tumors of the Central Nervous System. 4th ed. Lyon, France: IARC Press; 2007. p. 164-72
8. Fulkerson DH., Horner TG., Hattab EM. Histologically benign intraventricular meningioma.With concurrent pulmonary metastasis; case report and review of the literature. Clinical neurology and Neurosurgery. 2008-110: 416-419.
9. Zhiguo Liu, Chuanwei Wang, Hongwei Wang, Yunyan Wang, Jian Yi Li, Yuguang Liu: Clinical characteristics and treatment of angiomatous meningiomas: a report of 27 case.
10. Martin, H., Kay, W.N., Werner, P. 2004. Angiomatous meningioma clinicopathologic study of 38 cases. Am. J. Surg. Pathol., 28: 390–393.
11. Goyal R, Rathod G, Bhimani R, Gharia B. 2014. Oligodendroglioma in 40 years old male – A case report. Med. Sci., 9(33): 28–31.
12. Rao, S., Rajkumar, A., Kuruvilla, S. 2008. Angiomatous meningioma: A diagnostic dilemma. Ind. J. Pathol. Microbiol., 51(1): 53–55.
13. Rathod, G.B., Goyal, R., Yadav, S., Aggarwal, S., Goswami, SS. 2014. Intracranial hemangiopericytoma in 26 years old female – A case report. Med. Sci., 11(42): 47–5.
14. Rathod GB, Vyas K, Shinde P, Goswami SS and Tandan RK. Angiomatous meningioma in 49 years old male - A rare cas report Pathology :International Journal of Current Microbiology and Applied Sciences : 3 :11 (2014) pp. 256-260.
15. Hidehiro Takei,; Meenakshi B. Bhattacharjee, Andreana Rivera, Yeongju Dancer,; Suzanne Z. Powell,:New Immunohistochemical Markers in the Evaluation of Central Nervous System Tumors A Review of 7 Selected Adult and Pediatric Brain Tumors. Arch Pathol Lab Med. 2007;131:234–241.
16. Malak S. Abedalthagafi, et al. Angiomatous meningiomas have a distinct genetic profile with multiple chromosomal polysomies including polysomy of chromosome 5 Oncotarget. 2014; 5(21): 10596–10606.
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Source of support: Nil
Conflict of interest: None declared
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