CASE 18361 Published on 31.10.2023
0

Giant supratentorial cavernoma with internal haemorrhages

Section

Neuroradiology

Case Type

Clinical Cases

Authors

Lokesh Bommisetty, Palak Nandolia, Rahul Dev, Udit Chauhan, Khanak K. Nandolia

Department of Diagnostic and Intervention Radiology, All India Institute of Medical Sciences, Rishikesh, India

Connected authors
Khanakkumar Nandolia Neuroradiology Fellow India
Patient

25 years, male

Categories
Area of Interest CNS ; Imaging Technique CT, MR
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Clinical History

A 25-year-old male patient was admitted to the emergency services for evaluation of seizures involving left upper limb and lower limb with secondary generalization. He had dull headache, which gradually increased in severity over the period of last 6 months. On motor examination, he showed weakness of left upper and lower limbs with power of 2 out of 5.

Imaging Findings

Non-contrast CT (Figures 1 to 4) showed a large intra-axial heterogeneous space occupying lesion (white arrow) involving right cerebral hemispheres, with blood attenuation areas and calcifications. Vasogenic oedema was seen with midline shift toward left side and right sided uncal herniation (black arrow in Figure 3).

T2 and T2FLAIR images (Figures 5 to 8) showed a well-circumscribed intra-axial lesion in right frontal and parietal lobes with vasogenic oedema, measuring 8 centimetres in largest dimension.

The lesion showed internal multiple loculi with T2 hypointense areas and popcorn-like lobulated appearance at multiple places. DWI (Figure 9) and ADC map (Figure 10) showed restricted diffusion and low ADC value.

SWI and filtered phase images (Figures 11 and 12) showed blooming with mixed blood products and calcifications. Axial T1 (Figure 13) and T1FS (Figure 14) images showed T1 hyperintense areas within the lesion, not suppressing on FS. Post-contrast T1 fat-saturated image (Figure 15) showed no lesion enhancement.

Post-operative CT (Figure 16) showed a small post-operative cavity with pneumocephalus and reduction of mass effect.

Discussion

Background

Cavernoma or cavernous malformations are benign vascular lesions occurring in brain and spinal cord, with sizes ranging from few millimetres to few centimetres. They can undergo growth or involution. They can be sporadic or familial, congenital or acquired [1]. Giant cavernoma term is reserved for cavernoma larger than size of 6 centimetres [2]. Giant cavernoma poses a diagnostic challenge in imaging due to heterogeneous internal contents and mass effect.

Clinical Perspective

Cavernoma are benign vascular lesions composed of thin-walled, blood-filled, endothelial-lined capillary caverns. Supratentorial parenchyma is the most common location [1]. Cavernomas are angiographically occult. Complete surgical removal is for patients with symptomatic lesions associated with recurrent bleeds, seizures, and neurological deficits [1-3]. Familial cavernomas are associated with CCM1, CCM2 and CCM3 genes mutation. Acquired cavernoma can occur after radiotherapy to the brain. Cavernoma can remain silent. The incidence of a first symptomatic haemorrhage is 0.08% per patient per year. The risk of subsequent haemorrhage increases ten times after the first haemorrhage. Internal haemorrhages, increase in size and surrounding vasogenic oedema predispose a cavernoma to become symptomatic. No clear guidelines exist for surgical management of cavernoma. Consensus guidelines recommend surgical management after weighing benefits with risks of the surgery [3].

Imaging Perspective

Cavernomas are the second most common vascular malformation of the brain, followed by developmental venous anomalies, with 0.5–1% incidence in general populus [1], with even lower incidence of giant cavernoma [4,5]. Endothelial lining of the cavernoma forms an abnormal blood–brain barrier and hence allows chronic extravasation of blood products. This event forms the basis of imaging appearance of cavernoma with internal blood degradation products and hemosiderin staining in surrounding parenchyma – with typical rim of blooming. Imaging findings of cavernoma depend upon the composition of the lesion and stage of the blood products contained within the lesion. Common locations of cavernoma are cerebral hemispheres and brainstem.

On CT, cavernoma appears hyperdense due to blood products, calcifications or both. CT has sensitivity of 30–50% to detect intracranial cavernomas [1].

MR is the gold standard of imaging for cavernomas, recommended by Angioma Alliance, with class I evidence. Cavernoma imaging must include gradient echo (GRE) imaging [1,3]. Cavernomas show typical popcorn-like lobulated appearance with variable internal heterogeneous T1 and T2 signals depending upon the age of the blood products within. Surrounding parenchyma may show oedema and hemosiderin staining. Imaging checklist for a radiologist should describe number, locations and dimensions of lesions, mass effect and presence of other associated abnormalities. Cavernoma can be associated with an adjacent developmental venous anomaly (DVA).

Zabramski classification divides cavernoma into 4 types based on size, signal characters on T1 and T2 and morphology.

Outcome

Patient underwent craniotomy with surgical resection. The lesion showed well-circumscribed margins and a capsule. It was resected out en-toto. Internal appearance of the lesion was grape-like clusters filled with altered blood products and clots. Post-operative histopathology showed no components of malignancy. The lesion was confirmed to be a cavernoma.

Take Home Message

Giant cavernoma can mimic intra-axial neoplasms and may pose a diagnostic challenge.

Differential Diagnosis List
Giant cerebral cavernoma
Oligodendroglioma
Supratentorial ependymoma
Embryonal CNS tumour
Final Diagnosis
Giant cerebral cavernoma
Case information
URL: https://eurorad.org/case/18361
DOI: 10.35100/eurorad/case.18361
ISSN: 1563-4086
License

This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.

Figure 1

Axial non-contrast CT image – a large heterogeneous hyperdense lesion (white arrow) is seen in right cerebral hemisphere with internal specks of calcification and surrounding oedema.
Axial non-contrast CT image – a large heterogeneous hyperdense lesion (white arrow) is seen in right cerebral hemisphere with internal specks of calcification and surrounding oedema.
Figure 2

Axial non-contrast CT image – mass effect by the lesion with midline shift to left side (dashed line with arrow) and compression of both lateral ventricles.
Axial non-contrast CT image – mass effect by the lesion with midline shift to left side (dashed line with arrow) and compression of both lateral ventricles.
Figure 3

Coronal non-contrast CT image – mass effect by the lesion with midline shift to left side (dashed line), right uncal herniation (black arrow) and compression of both lateral ventricles.
Coronal non-contrast CT image – mass effect by the lesion with midline shift to left side (dashed line), right uncal herniation (black arrow) and compression of both lateral ventricles.
Figure 4

Sagittal non-contrast CT image – lesion (white arrow) with internal specks of calcification and surrounding oedema.
Sagittal non-contrast CT image – lesion (white arrow) with internal specks of calcification and surrounding oedema.
Figure 5

Axial T2W MR image – lobulated well-circumscribed lesion in right cerebral hemisphere with internal bubbly multiloculated appearance and mixed signal on T2. Midline shift to left is seen. Lesion has a hypointense rim.
Axial T2W MR image – lobulated well-circumscribed lesion in right cerebral hemisphere with internal bubbly multiloculated appearance and mixed signal on T2. Midline shift to left is seen. Lesion has a hypointense rim.
Figure 6

Axial T2 FLAIR MR image – internal bubbly appearance and mixed signal on T2 with partial suppression of fluid contents. Surrounding vasogenic oedema is seen.
Axial T2 FLAIR MR image – internal bubbly appearance and mixed signal on T2 with partial suppression of fluid contents. Surrounding vasogenic oedema is seen.
Figure 7

Sagittal T2W MR image – T2 hypointense lesion rim and internal blood fluid levels in few loculi are well demonstrated.
Sagittal T2W MR image – T2 hypointense lesion rim and internal blood fluid levels in few loculi are well demonstrated.
Figure 8

Coronal T2W MR image – T2 hypointense lesion rim and midline shift with ventricular compression are demonstrated.
Coronal T2W MR image – T2 hypointense lesion rim and midline shift with ventricular compression are demonstrated.
Figure 9

Axial DWI MR image – restricted diffusion in few loculi of the lesion.
Axial DWI MR image – restricted diffusion in few loculi of the lesion.
Figure 10

Axial ADC image – low ADC value in few loculi of the lesion.
Axial ADC image – low ADC value in few loculi of the lesion.
Figure 11

Axial SWi image – low signal in most of the part of the lesion with striking hypointense rim and lobulated popcorn-like outline.
Axial SWi image – low signal in most of the part of the lesion with striking hypointense rim and lobulated popcorn-like outline.
Figure 12

Axial filtered phase image – mixed signal in the lesion denoting coexisting blood products and calcifications in various lesion components.
Axial filtered phase image – mixed signal in the lesion denoting coexisting blood products and calcifications in various lesion components.
Figure 13

Axial T1W image – predominantly bright signal within the lesion with few isointense areas.
Axial T1W image – predominantly bright signal within the lesion with few isointense areas.
Figure 14

Axial T1W fat-saturated image – predominantly bright signal within the lesion with no areas of suppression.
Axial T1W fat-saturated image – predominantly bright signal within the lesion with no areas of suppression.
Figure 15

Axial T1W fat-saturated post-contrast image – no internal or peripheral component of enhancement.
Axial T1W fat-saturated post-contrast image – no internal or peripheral component of enhancement.
Figure 16

Axial non-contrast post-operative CT image – resection cavity at the previous lesion site and relief of mass effect due to lesion. Post-operative pneumocephalus is seen.
Axial non-contrast post-operative CT image – resection cavity at the previous lesion site and relief of mass effect due to lesion. Post-operative pneumocephalus is seen.