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Year : 2010  |  Volume : 6  |  Issue : 4  |  Page : 560-563

Cystic angiomatous meningioma in the cerebellopontine angle mimicking hemangioblastoma

1 Department of Pathology, Armed Forces Medical College, Pune, India
2 Department of Radiology, Armed Forces Medical College, Pune, India
3 Department of Neurosurgery, Command Hospital, Pune, India

Date of Web Publication24-Feb-2011

Correspondence Address:
Prabal Deb
Associate Professor and Neuropathologist, Department of Pathology, Armed Forces Medical College, Pune
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.77074

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 > Abstract 

We hereby report an extremely unusual case of cystic angiomatous meningioma in the CPA region in a 58-year-old male patient. He presented with complaints of headache, repeated episodes of vomiting and increasing unsteadiness of gait. Neuroimaging showed a large multicystic left-sided tentorial tumor projecting into the cerebellum and CPA with contrast-enhancing peripheral solid rim. He underwent a left retromastoid craniectomy and total excision of the tumor. Histopathology revealed an angiomatous meningioma with predominant microvascular component and extensive cystic changes. Immunopositivity for epithelial membrane antigen (EMA), vimentin and S-100 protein proved vital in excluding a hemangioblastoma.

Keywords: Angiomatous, cerebellopontine angle, cystic, meningioma

How to cite this article:
Deb P, Sahni H, Bhatoe HS. Cystic angiomatous meningioma in the cerebellopontine angle mimicking hemangioblastoma. J Can Res Ther 2010;6:560-3

How to cite this URL:
Deb P, Sahni H, Bhatoe HS. Cystic angiomatous meningioma in the cerebellopontine angle mimicking hemangioblastoma. J Can Res Ther [serial online] 2010 [cited 2022 Jun 26];6:560-3. Available from: https://www.cancerjournal.net/text.asp?2010/6/4/560/77074

 > Introduction Top

Cystic meningiomas are uncommon tumors that have predilection for the pediatric age group, and commonly occur in the cerebral convexity and parasaggital areas. These neoplasms often mimic gliomas, metastatic malignancies, or hemangioblastomas, which are more frequently associated with a cystic morphology. [1],[2],[3]

Angiomatous meningiomas are also rare and its association with cystic morphology, unlike meningothelial type, is uncommon. Most angiomatous meningiomas affect the cerebral convexity, and its occurrence in the cerebellopontine angle (CPA) is unusual. [1],[4] Encountering a meningioma with both cystic and angiomatous pattern is exceptional, and its location in the CPA region, to the best of our knowledge, has never been reported.

 > Case Report Top

A 58-year-old male patient presented with complaints of headache, repeated episodes of vomiting and increasing unsteadiness of gait. Clinical examination revealed left-sided cerebellar dysmetria with dysdiadochokinesia. He was normotensive and lacked any neurocutaneous marker. Routine laboratory investigations were within normal limits.

Computerized tomography (CT) scan showed a cystic tumor in the left CPA, close to the tentorium. Contrast studies showed rim enhancement. There was no hydrocephalus or calcification. Magnetic resonance imaging (MRI) revealed a large multicystic left tentorial tumor measuring 5 × 4 × 3 cm with solid mural component, projecting into the left cerebellum and CPA. There was prominent enhancement of the internal septae and the eccentric solid mural component [[Figure 1]a-c].
Figure 1: T1 weighted MR images with gadolinium in axial (a), saggital (b) and coronal plane (c), showing a left tentorial based, infra tentorial cystic mass with solid mural component. The mass measures 56 × 41 × 34 mm. There is mass effect with midline shift. There is prominent enhancement of the internal septae and the eccentric solid mural component. The transverse sinus is not infiltrated. Minimal perilesional edema is seen in the adjoining cerebellar hemisphere

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The transverse sinus is not infiltrated. Minimal perilesional edema is seen in the adjoining cerebellar hemisphere.

A left retromastoid craniectomy with total excision of the tumor was performed. Peroperatively, dura was defined between lateral and sigmoid sinuses. On retracting the cerebellum, a firm, well defined cystic vascular tumor containing straw colored fluid was noted, which was adherent to tentorial leaf and compressing the cerebellum, which could be excised completely.

Tissue was fixed in 10% neutral buffered formalin, routinely processed and paraffin embedded. Five-micron thick sections were cut and stained by hematoxylin and eosin (H and E) stain. Immunohistochemical staining was done by streptavidin-biotin immunoperoxidase technique (LSAB Kit, M/s Dakopatts, Denmark) using monoclonal antibodies to epithelial membrane antigen (EMA), vimentin (Vim), S-100 protein, and glial fibrillary acidic protein (GFAP); while proliferation index was evaluated using MIB-1 antibody staining (all antibodies were prediluted and obtained from M/s BioGenex, US).

Microscopically, tumor appeared highly vascular with prominent cystic areas. Majority of the tumor was composed of small, capillary-like and thin-walled blood vessels ('microvascular pattern'). Intervening areas contained variable amount of tumor cells with vacuolated cytoplasm [[Figure 2]a-d]. There was no increased mitosis, microvascular proliferation or necrosis.

Immunopositivity of tumor cells for epithelial membrane antigen (EMA), vimentin [[Figure 2]e, f] and S-100 protein confirmed a diagnosis of angiomatous meningioma in preference to hemangioblastoma.
Figure 2: Photomicrograph showing a cystic tumor at the cerebellopontine angle (a: Hand E, ×40), consisting of large cystic spaces lined by meningothelial component (b: H and E, ×40) and widespread areas of small, capillary type thin-walled vessels ('microvascular pattern') (c: H and E, ×100) interspersed with foamy cells with vacuolated cytoplasm (d: H and E, ×200). Tumor cells showed immunopositivity for EMA (e: EMA ×200) and Vim (f: Vim ×200)

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MIB-1 labeling index (MIB-LI) was < 1%.

Post-operative recovery was largely uneventful. He was on regular follow-up for the next six months; and at last review, he was asymptomatic, ambulant, without any neurological deficit.

 > Discussion Top

Meningiomas comprise 15-18% of intracranial tumors in adults and 33% of all incidental intracranial neoplasms. [1] Angiomatous meningioma is an uncommon variant accounting for 2.1% of all meningiomas and having predilection for the cerebral convexity. [4]

In the CPA, meningiomas account for approximately 3-13% of all neoplasms, with two large series reporting an incidence of 6.5%. [5] The most common variants are meningothelial and transitional, while angiomatous is a rarity. [5],[6] It is postulated that CPA meningiomas originate from the cells lining arachnoid villae located adjacent to intracranial veins and dural sinuses. They usually grow from areas adjacent to the sigmoid sinus, jugular foramen, torcula, or the superior and inferior petrosal sinuses. [5]

Cystic meningiomas are also rare, accounting for 0.7% to 11.7% of all meningiomas. [2] These neoplasms generally affect children and have predilection for convexity and parasaggital sinus, features which were contrary to the present case. It is unusual for cystic meningiomas to either display an angiomatous component or to occur in the CPA; [7] and meningiomas in the CPA region to have both cystic and angiomatous pattern is not known.

Cyst formation in meningiomas is thought to be a resultant of ischemic necrosis, cystic degeneration, intratumoral hemorrhage, trapping of CSF, peritumoral edema into cyst, active secretion by tumor cells, or due to glial reaction and transudation. [8] Cystic meningiomas have been classified by Nauta et al[9] into types 1 to 4, based on if cysts were intratumoral, peritumoral with / without tumor lining cyst wall, and cyst at the brain-tumor interface. The present case was of type 2. With the availability of CT scan and MRI, the incidence of diagnosing cystic meningioma has increased. Currently, MRI especially with gadolinium enhancement, is the preferred mode, since this is able to distinguish cyst wall invaded by tumor cells (type 2) from cyst wall composed of gliotic tissue without tumor infiltration (type 3). The presence of a nodule of a cystic lesion close to the dura, and enhancement of the adjacent dura suggests the diagnosis of a cystic meningioma. [3],[10]

In general, 'true' intratumoral cysts in association with highly vascularised meningiomas are exceedingly rare, with an incidence of around 0.2% only. [7] In the past, most cases of highly vascularized cystic meningiomas have been reported as 'angioblastic meningioma', an obsolete term which has often included hemangioblastoma and hemangiopericytoma, apart from transitional cases between meningioma and hemangioblastoma, vascular malformation and metastasis from renal cell carcinoma. [1],[7]

On histopathology, angiomatous meningioma is chararcterised by abundance of well-formed vascular channels, sinusoids or capillaries. In their series of 38 cases, Hasselblatt et al[4] noted two subtypes of angiomatous meningioma, viz. microvascular subtype (more than 50% contain vessels with diameter below 30 μ), and a macrovascular subtype. These tumors may also display microcystic change along with foamy cells (which are related to leakage of plasma lipids across thin vessel walls); in addition to solid areas with meningotheliomatous meningioma elements. In view of the cystic neoplasm on imaging, prominent microvascular pattern with foamy cells in the present case suggested the possibility of hemangioblastoma.

Hemangioblastomas are benign neoplasms of uncertain histogenesis constituting 1-2% of all primary central nervous system tumors. [1] Cerebellar hemangioblastoma is the most common initial manifestation, affecting 64% of patients with von Hippel-Lindau (VHL) disease. MRI has largely replaced both angiography and CT scan. Currently gadolinium-enhanced MRI, showing a contrast-enhancing nodule with the associated cyst or syrinx, is the diagnostic method of choice. Although false-negatives may be seen with small lesions (<5 mm) and with delayed imaging, false-positive findings are rare and may be due to metastatic lesions or arachnoid cysts. [11] On gross the lesions are small nodules in the wall of a cyst. The fluid is characteristically yellow to brown, whereas the tumor is red and / or yellow depending on the vascularity and lipid content. On histopathology, large vacuolated stromal cells along with a rich capillary network characterize hemangioblastoma. The 'clear cell morphology' of stromal cells due to numerous lipid-containing vacuoles mimics metastatic renal cell carcinoma, while the exuberant gliosis around the cyst wall may resemble pilocytic astrocytoma. [1]

Since both angiomatous meningioma and hemangioblastoma are benign tumors, the need to differentiate them from therapeutic viewpoint appears irrelevant, though a confirmed diagnosis of hemangioblastoma often suggests VHL disease. Further, differentiating angiomatous meningiomas from hemangiopericytoma is imperative since the former is not associated with aggressive clinical behavior. [1],[7]

 > References Top

1.Perry A, Louis DN, Scheithauer BW, Budka H, Von Deimling A. Meningiomas. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, editors. World Health Organization classification of tumors of the central nervous system, 4 th ed. Lyon: International Agency for Research on Cancer (IARC) Press; 2007. p. 164-77.   Back to cited text no. 1
2.Özer E, Yucesoy K, Citak GL, Koyuncuoglu M. Cystic Meningioma Imitating Vestibular Schwannoma at the Cerebellopontine Angle: Case Report. J Neurologi Sci (Turkish) 2007;24:84-7.  Back to cited text no. 2
3.Chen TY, Lai PH, Ho JT, Wang JS, Chen WL, Pan HB, et al. Magnetic resonance imaging and diffusion-weighted images of cystic meningioma: Correlating with histopathology. Clin Imaging 2004;28:10-9.  Back to cited text no. 3
4.Hasselblatt M, Nolte KW, Paulus W. Angiomatous meningioma: A clinicopathologic study of 38 cases. Am J Surg Pathol 2004;28:390-3.  Back to cited text no. 4
5.Carney AS, Ward V, Malluci CL, O'Donoghue GM, Robertson I, Baldwin DL, et al. Meningioma involving the internal auditory canal: A diagnostic and surgical challenge. Skull Base Surg 1999;9:87-93.  Back to cited text no. 5
6.Thomas NW, King TT. Meningiomas of the cerebellopontine angle. A report of 41 cases. Br J Neuro surg 1996;10:59-68.  Back to cited text no. 6
7.Taraszewska A, Bogucki J. A case of cystic form of angiomatous meningioma with prominent microvascular pattern mimicking haemangioblastoma. Folia Neuropathol. 2001;39:119-23.  Back to cited text no. 7
8.Zee CS, Chen T, Hinton DR, Tan M, Segall HD, Apuzzo ML. Magnetic resonance imaging of cystic meningiomas and its surgical implications. Neurosurg 1995;36:482-8.   Back to cited text no. 8
9.Nauta HJ, Tucker WS, Horsey WJ, Bilbao JM, Gonsalves C. Xanthochromic cysts associated with meningioma. J Neurol Neurosurg Psychiatry 1979;42:529-35.   Back to cited text no. 9
10.Hu SL, Li F, Hu R, Cui G, Meng H, Feng H.none Atypical histopathologic type of cystic meningioma. Acta Neurochir (Wien)none 2010;152:105-9.  Back to cited text no. 10
11.Vatsal D, Husain M, Husain N, Chawla S, Roy R, Gupta RK. Cerebellar hemangioblastoma simulating arachnoid cyst on imaging and surgery. Neurosurg Rev 2002;25:107-9.  Back to cited text no. 11


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