Dura-based cavernous hemangioma presenting as large intracerebral hematoma in a child: A rare clinico-pathological entity.

Dear Sir,

Cavernous hemangiomas aka cavernous angiomas aka cavernomas are angiographically occult vascular malformation, which most often present as intracerebral hematoma or seizures in young adults.[1] These are second only in frequency of occurrence to developmental venous anomalies (DVT) and equal in that of cerebral arterio-venous malformations.[1-3] Dura-based cavernoma hitherto is a rare clinico-pathological entity affecting the central nervous system.[4-6] We present a 13-year-old girl who presented with intracerebral hematoma secondary to a temporal convexity dura-based cavernoma.

A 13-year-old girl presented with sudden onset of headache and vomiting. The headache was severe in intensity, localized to the right temporal region, and referred to the right eye. There was no history of loss of consciousness, seizures, diplopia, speech or memory disturbance, and limb weakness. On examination she had no focal neurological deficit except for mild upward gaze restriction. Initial CT brain showed a 7 × 5 cm right temporal intracerebral hematoma with mass effect and midline shift of 0.4 cm. MRI brain with GADO was done which showed an acute-to-subacute hematoma with a dura-based lesion with contrast enhancement [Figure 1]. Six-vessel cerebral DSA showed tumor blush with feeding vessel arising from the right middle meningeal artery and there was no evidence of arterio-venous malformation [Figure 2]. In view of significant mass effect and suspected underlying pathology it was decided to do craniotomy, evacuation of intracerebral hematoma, and excision of the dura-based lesion. Right fronto-temporal craniotomy was performed and on opening the dura, a “mulberry-red” lesion was found attached to the convexity dura. It was predominantly extra-axial with a protruding portion dipping into the hematoma cavity. The tumor was resected completely along with its dural attachment and the hematoma evacuated [Figure 3]. The patient made an uneventful recovery following the surgery with no deficits. The histo-pathological study showed multiple sinusoidal spaces filled with blood lined by thin endothelium cells, consistent with cavernous angioma [Figure 4].

Figure 1

MRI brain and MRA. (a) and (b) Axial clear T1WI and T2WI showing a large right temporal heterointense lesion which is predominantly isointense on T1WI and T2WI and it has a peripheral zone that is hyperintense on T1WI and T2WI suggestive of acute clot. (c) MRI with GADO showing enhancement of the dura-based lesion along the temporal convexity

Figure 2

Digital subtraction angiogram of right external carotid injection (oblique view) showing feeding arteries arising from main trunk of middle meningeal artery

Figure 3

Above is an intraoperative picture of the dura-based lesion with a portion dipping into the hematoma cavity. Below is a biopsy specimen with dural attachment

Figure 4

H and E, ×4. Lesion near the dural attachment. The dura mater is seen as a fibro-collagenous layer on the inferior aspect of slide. Inset shows ×10 magnification with multiple small single-cell-layered sinusoidal spaces filled with blood consistent with cavernous angioma

Cavernous angiomas or cavernomas are second only to arteriovenous malformations presenting in children. They show no sexual preponderance. They have a silent course and a sizeable number of cavernoma is picked up incidentally. Seizures and hemorrhage are the two common modes of clinical manifestation. Radiological appearance on CT brain is often due to the presence of blood in and around the lesion. On MRI, the lesion has a characteristic “pop corn” appearance due to the presence of varying stages of blood degradation products. Histologically cavernoma is formed from blood filled sinusoidal space lined by endothelium devoid pericytes, smooth muscles or elastic fibers.[7] Cavernous angioma can occur in any part of the body and the central nervous system.[8] The locations in the order of highest to lowest frequency are cerebral hemispheres, brain stem, spinal cord, and extra-axial location.[910] Infrequent locations such as skull base dural angiomas in the cavernous sinus,[1112] intraventricular cavernoma,[13] optico-chiasmatic cavernoma,[14] and orbital cavernoma[15] have been reported. Posterior fossa dura-based cavernoma arising from superior petrosal sinus was reported by Kocak et al., in 2002,[8] and a lesion arising from cerebellar falx by Ito et al., in 2009.[5] McCormick in 1966 had described angiomas of dura mater.[16] Hitherto a supratentorial convexity dura-based cavernous angioma is a very rare entity.[6] Dura-based parietal convexity cavernoma mimicking meningioma was reported in a 77-year-old lady who presented with seizure by Perry et al., in 1993.[4] Joshi et al. have recently (2009) reported a supratentorial convexity dura-based cavernoma in a 15-year-old boy who presented with headache and showed good results following surgical excision. Dura-based cavernous angioma presenting with large intracerebral hematoma is an extremely rare case. As with intracerebral cavernoma surgical excision gives complete and lasting cure in these patients.