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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 10  |  Issue : 1  |  Page : 60-62

An intracerebral sporadic mixed cavernous angioma: An extremely rare entity with review of literature


Department of Pathology, Dr. D Y Patil Medical College, DPU, Pune, Maharashtra, India

Date of Submission08-Sep-2022
Date of Decision09-Sep-2022
Date of Acceptance10-Sep-2022
Date of Web Publication22-Sep-2022

Correspondence Address:
Dr. Shirish Sahebrao Chandanwale
Department of Pathology, Dr. D Y Patil Medical College, DPU, Pune, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jcvs.jcvs_25_22

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  Abstract 


Cerebral vascular malformations are non-neoplastic lesions resulting from focal anomalies in the development of cerebrospinal circulation. They tend to be asymptomatic, but in 40% of cases, they can be symptomatic; the four morphological types have been described in standard surgical pathology textbooks such as capillary, cavernous, venous and arteriovenous malformations. Mixed types are very rare and remain exceptional. Computed tomography (CT) and CT angiography lack sensitivity and specificity for accurate diagnosis. Magnetic resonance imaging is the choice of diagnostic modality. A conservative approach is observed in asymptomatic patients. Microsurgical resection is the only definitive treatment in symptomatic patients for supratentorial lesions. Due to the heterogeneity of this entity and ongoing debate about ontogenesis, diagnosis and management, we report the case of an 18-year-old boy who presented with a history of seizures for 3 years.

Keywords: Angioma, cavernoma, microsurgery, mixed


How to cite this article:
Chandanwale SS, Randive RS, Rashmi RK, Ravishankar R, Yadav SR, Patel N. An intracerebral sporadic mixed cavernous angioma: An extremely rare entity with review of literature. J Cerebrovasc Sci 2022;10:60-2

How to cite this URL:
Chandanwale SS, Randive RS, Rashmi RK, Ravishankar R, Yadav SR, Patel N. An intracerebral sporadic mixed cavernous angioma: An extremely rare entity with review of literature. J Cerebrovasc Sci [serial online] 2022 [cited 2022 Oct 4];10:60-2. Available from: http://www.jcvs.com/text.asp?2022/10/1/60/356706




  Introduction Top


Vascular malformations of the central nervous system are seen increasingly in clinical practice, because of advances in cross-sectional brain and spinal cord imaging. Cerebral vascular malformations are non-neoplastic lesions resulting from focal anomalies in the development of cerebrospinal circulation. Many surveys suggest that cavernous angiomas are present in 0.5% of population. They usually tend to be asymptomatic, but in 40% of cases, they can be symptomatic.

The four morphological types have been described in standard surgical pathology textbooks, namely, (1) capillary telangiectasia, (2) cavernous angioma or cavernoma, (3) venous angioma and (4) arteriovenous malformations. Cavernous angiomas are uncommon, but they account for 10%–25% of all vascular malformations. Mixed cavernous angiomas are very rare and remain exceptional. They can be either familial or sporadic.[1],[2] Understanding of natural history of this entity is of great importance to avoid unnecessary morbid interventions. We present this case due to the heterogeneity of this entity and ongoing debate about ontogenesis, diagnosis and management.[3]


  Case Report Top


An 18-year-old male came to the neurosurgery outpatient department with a history of seizures for 3 years. The latest episode was on 1 month back. He did not give any other major past history of illness. There was no family history of similar illness. A thorough clinical examination did not reveal any neurological deficit. His vital parameters were within normal limits. The patient underwent plain axial computed tomography (CT) scan, and the report revealed a cortex-based lesion in the parafalcine right anterior frontal region measuring 3 cm × 3 cm × 3 cm involving the subcortical region. The lesion was heterogeneous and showed mixed attenuation with multiple small calcific and haemorrhagic foci. Neoplastic aetiology was suspected. Subsequent magnetic resonance imaging (MRI) revealed a well-defined heterogeneously hyperintense lesion at the same site measuring about 3.1 cm × 3.1 cm × 3 cm with a surrounding rim of haemosiderin deposition and internal haemorrhagic and calcific foci on T2-weighted images [Figure 1]a. A diagnosis of vascular malformation was made.
Figure 1: (a) MRI showing a heterogeneous cortex-based lesion, (b) Vascular lumen with thick fibrous wall and calcification (Leishman stain, ×400), (c) Adjacent brain parenchyma (Leishman stain, ×100), (d) Areas with capillaries (arrow, Leishman stain, ×100). MRI: Magnetic resonance imaging

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The patient was operated and underwent microsurgical resection. Multiple greyish-black fragmented bits of tissue were received for histopathological examination. The tissue was fixed in 10% formalin and paraffin processed. The 3–4 μ thick sections were cut and stained with haematoxylin and eosin (H and E). Histopathology examination showed closely opposed vascular channels made up of hyaline fibrous wall with lining of a single layer of endothelial cells and full of blood. There was no intervening cerebral parenchyma. Few areas of dystrophic calcification and ossification were seen. Normal cerebral parenchyma was noted at the periphery of the lesion [Figure 1]b and [Figure 1]c. Some areas showed prominent congested capillaries [[Figure 1]d, arrow]. Histochemical stains such as Verhoeff's and Masson's trichrome stains showed the absence of elastic tissue and muscle in vessel walls, respectively [Figure 2]a and [Figure 2]b. A histopathology diagnosis of cavernous angioma mixed or hybrid type was made.
Figure 2: Cavernous angioma (a) (Verhoeff's stain, ×400), (b) (Masson's trichrome stain, ×400)

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  Discussion Top


The cavernous angiomas are also called cavernomas, and they occur in both children and adults with the mean age of the patient in the fourth decade. There is no gender predilection.[4],[5] Majority of the cavernous malformations are sporadic, but 20% of cases may be familial. Sporadic cases often occur as a single lesion. The familial cavernous angiomas are often multiple and caused by mutations in CCM1, CCM2 and CCM3 genes. They are inherited as autosomal dominant.[3],[6] In our case, there was no family history of similar illness.

The most common location is supratentorial region in the cerebrum though they can occur in basal ganglia, brainstem, cerebellum and spine. The signs and symptoms depend on the size and location of the lesion. They commonly present clinically with headache, seizures, focal neurological deficit, intracerebral haemorrhage or they could be asymptomatic.[7] Cavernous angiomas that are supra-tentorial frequently present with seizures, while those that are infra-tentorial present with focal neurological deficit or ataxia.[5] Seizures as only presentation in our case can be attributed to the proximity of the lesion to epileptogenic cerebrocortical tissue.

CT scan and CT angiography have a limited role in diagnosis because it lacks sensitivity and specificity for accurate diagnosis. In our case, similar observations were made. It typically shows a lesion with heterogeneous core typically surrounded by a low-signal-intensity haemosiderin rim.[6] MRI is the choice of diagnostic modality.[3]

In our case, histopathology examination showed a mixture of vascular channels with thick hyaline fibrous walls and areas with capillaries. The diagnosis of mixed or hybrid type was given. The histological features along with the absence of muscle and elastic tissue in the vessel wall in Masson's trichrome and Verhoeff's stains ruled out the possibility of arteriovenous malformation.

Existence of mixed type angiomas, in which vessels with hyalinised fibrous walls and capillaries in the same lesion, have created speculation that they may evolve from capillary telangiectasia. In contrast, some authors believe microhaemorrhages around cavernous haemangioma initiate processes of reactive angiogenesis which lead to the formation of a mixed form of malformations.[8],[9] In our case, capillaries were seen in the centre of the lesion but not at the periphery of the lesion.

A conservative approach is observed in asymptomatic cavernous angiomas. Microsurgical resection is the only definitive treatment in symptomatic patients for supratentorial lesions. It has a high success rate with relatively low complications. Similar observations were made in our case. On follow-up of the patient after 1 year, he did not experience any symptoms.


  Conclusion Top


Cavernous angiomas are uncommon lesions. Signs and presenting symptoms depend on the size and location of the lesions. CT findings are non-specific. MRI is the choice of diagnostic modality. The presence of both vessels with hyalinised fibrous walls and capillaries in the same lesion suggests a mixed or hybrid type which is very rare. A conservative approach is used to treat asymptomatic cases. Microsurgical resection is the choice of treatment in symptomatic patients.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Rosai and Ackerman's Surgical Pathology. 10th ed. Mosby, Missouri: An Imprint of Elsevier; 2012. p. 1957-58.  Back to cited text no. 1
    
2.
Mouchtouris N, Chalouhi N, Chitale A, Starke RM, Tjoumakaris SI, Rosenwasser RH, et al. Management of cerebral cavernous malformations: From diagnosis to treatment. ScientificWorldJournal 2015;2015:808314.  Back to cited text no. 2
    
3.
Flemming KD, Link MJ, Christianson TJ, Brown RD Jr. Prospective haemorrhage risk of intracerebral cavernous malformations. Neurology 2012;78:632-6.  Back to cited text no. 3
    
4.
Dalyai RT, Ghobrial G, Awad I, Tjoumakaris S, Gonzalez LF, Dumont AS, et al. Management of incidental cavernous malformations: A review. Neurosurg Focus 2011;31:E5.  Back to cited text no. 4
    
5.
Washington CW, McCoy KE, Zipfel GJ. Update on the natural history of cavernous malformations and factors predicting aggressive clinical presentation. Neurosurg Focus 2010;29:E7.  Back to cited text no. 5
    
6.
Horne MA, Flemming KD, Su IC, Stapf C, Jeon JP, Li D, et al. Clinical course of untreated cerebral cavernous malformations: A meta-analysis of individual patient data. Lancet Neurol 2016;15:166-73.  Back to cited text no. 6
    
7.
Ene C, Kaul A, Kim L. Natural history of cerebral cavernous malformations. Handb Clin Neurol 2017;143:227-32.  Back to cited text no. 7
    
8.
McCormick WF. The pathology of vascular (”arteriovenous”) malformations. J Neurosurg 1966;24:807-16.  Back to cited text no. 8
    
9.
Karri SB, Uppin MS, Rajesh A, Ashish K, Bhattacharjee S, Rani YJ, et al. Vascular malformations of central nervous system: A series from tertiary care hospital in South India. J Neurosci Rural Pract 2016;7:262-8.  Back to cited text no. 9
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