Cavernous haemangioma in the interpeduncular cistern: case report and review of literature.
DP Muzumdar, MG Bhatjiwale, A Goel, P Doshi
Department of Neurosurgery, Seth G. S. Medical College and King Edward VII Memorial Hospital, Mumbai, India. , India
D P Muzumdar
Department of Neurosurgery, Seth G. S. Medical College and King Edward VII Memorial Hospital, Mumbai, India.
A rare case of a cavernous haemangioma in the interpeduncular cistern is reported. The patient, forty-five year old male presented with excruciating left sided trigeminal neuralgia and diplopia for the past one year. Examination revealed left third and fifth nerve paresis. Magnetic resonance imaging showed a well-defined, lobulated tumour in the interpeduncular cistern. The tumour was totally excised through a subtemporal route. Histology of the tumour revealed a cavernous haemangioma. Extracerebral location for a cavernous haemangioma is rare. An interpeduncular cavernous haemangioma has never been reported earlier in literature. The clinical and radiological features are discussed and relevant literature is briefly reviewed.
|How to cite this article:|
Muzumdar D P, Bhatjiwale M G, Goel A, Doshi P. Cavernous haemangioma in the interpeduncular cistern: case report and review of literature. J Postgrad Med 2001;47:191-3
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Muzumdar D P, Bhatjiwale M G, Goel A, Doshi P. Cavernous haemangioma in the interpeduncular cistern: case report and review of literature. J Postgrad Med [serial online] 2001 [cited 2021 Apr 21 ];47:191-3
Available from: https://www.jpgmonline.com/text.asp?2001/47/3/191/196
Extracerebral cavernomas are commonly reported in the middle fossa in proximity to the cavernous sinus. Cavernous haemangioma is reported in the posterior fossa dura, cerebellopontine angle, cavernous sinus. A cavernous haemangioma involving the interpeduncular cistern has not been reported earlier. The clinical presentation and the radiological features may resemble a meningioma or schwannoma as was seen in our case.
A forty-five year old male presented with gradual onset progressive left fronto-temporal headache and facial pain for the past one year. The left facial pain was neuralgic, mainly over the cheek and jaw. The patient also noticed progressive diplopia, ptosis along with excessive lacrimation in the left eye. Examination revealed a left third nerve and fifth nerve sensory paresis. Vision was 6/18 in both eyes. Rest of the neurological examination was unremarkable. He had multiple pinhead sized cutaneous angiomas all over the body. Magnetic resonance imaging (MRI) of the brain showed irregular lobulated tumour in the interpeduncular cistern [Figure:1]. It was moderately hyperintense on T1 and T2-weighted images with a hypointense peripheral rim. Angiography revealed an avascular mass. The patient underwent a left subtemporal craniotomy. The tumour was found to lie adjacent to the tentorial edge. The oculomotor nerve was stretched over it. The lower part of the lesion was seen to be in contact with the root entry zone of the fifth nerve. The lesion was lobulated, reddish brown and the surrounding structures showed xanthochromia. Dissection of the tumour from the surrounding structures was not difficult since it was not adherent to the surrounding blood vessels. The oculomotor nerve was greatly attenuated and was inadvertently injured during the dissection. The medial part of the tumour was in close proximity to the basilar artery. There was no obvious evidence of any vascular supply to the tumour. It could be separated away from the basilar artery with relative ease. Total excision of the tumour was done. Postoperatively, the patient had a left third nerve palsy but an otherwise uneventful recovery. He had complete relief in his neuralgic pain and headaches. Postoperative MRI of the brain showed no evidence of tumour. Histological examination showed proliferating blood vessels of varying calibre with thin fibrous septa as well as vascular spaces lined by delicate endothelium and filled with red blood corpuscles [Figure:2]. Histological features suggested a diagnosis of cavernous haemangioma. At follow-up after three years, the patient is well and asymptomatic.
Cavernous haemangiomas are hamartomas representing 5% to 13% of all the central nervous system vascular malformations. They most commonly involve the cerebral hemispheres, especially the parietal lobe and the basal ganglia. Extra-axial location of cavernous haemangioma comprises about 13% of all haemangiomas in the brain. Out of this 73% arise from the dura of the middle fossa/cavernous sinus. Interpeduncular cistern is an extremely unusual location for a cavernous haemangioma. We were unable to locate a similar case in literature.
The clinical features are non-specific for the diagnosis of a cavernoma. The involvement of the third and the fifth nerve suggests that the tumour was occupying a significant portion in the interpeduncular cistern. The irritation of the root entry zone of the trigeminal nerve, in our case is also unusual. The commonest tumour in this location would be a meningioma or a schwannoma. Angiography did not reveal any vascular lesion or any abnormal circulation in the tumour in our case. Until recently, the diagnosis of cavernomas was retrospect. The initial use of plain cranial radiography for the evaluation of cavernous haemangiomas is of historical interest only. CT has a sensitivity of 70% to 100% but the specificity is quite poor (< 50 %). CT can observe haematoma or calcification but frequently does not delineate the underlying lesion or differentiate the lesion type. Angiography usually reveals an avascular mass. Hypervascularity, venous pooling, blush and neovascularisation can be seen. MR imaging of cavernous haemangiomas is characteristic and is immensely helpful in its preoperative evaluation and aids in surgical management. It shows a well-circumscribed mass with mixed intensity or non-homogeneous areas of iso to hypointensity surrounded by a hypointense ring suggestive of haemosiderin deposition. The appearance of the central core is generated by blood by-products in different stages of evolution. Contrast enhancement usually suggests presence of abnormal or anomalous veins. Venous angioma may coexist with a cavernous haemangioma. A cavernoma is characterised histologically by lack of intervening brain parenchyma. There is a characteristic gliomatous reaction of the surrounding parenchyma, which may form a capsule around the lesion. Hyalinisation, thrombosis, calcification, cysts and cholesterol crystals are seen within the lesion.
Total excision using microsurgical techniques is ideal since cavernoma is well circumscribed and lacks attachment to the surrounding brain. The extra-axial cavernomas have a higher incidence of profuse intraoperative bleeding and spectrum of postoperative haematoma than intraxial cavernomas. There is a high risk (20-80%) of recurrent haemorrhage. Early surgical intervention is mandatory since progressive neurological decline occurs after initial bleeding from a cavernoma. The annual clinically significant risk of haemorrhage has been estimated at 0.7% to 1.1% per lesion per year. The haemorrhage is invariably secondary to the cavernoma in mixed lesions comprising of a cavernoma and a venous malformation. The risk of haemorrhage is eliminated permanently by surgery. Radiosurgery for cavernous malformations of the brain has a poor clinical response and high complication rate with the standard doses used for an arterio-venous malformation. The long-term risks and safe-dose levels need to be further evaluated and a prolonged clinical follow-up is required. A significant decrease in the bleeding rate has been found more than 3 years after treatment compared with the bleeding rate within 3 years of treatment.
To conclude, MRI is an ideal investigation for diagnosis of cavernoma. A mixed signal intensity mass with a surrounding rim of haemosiderin on T2-weighted image is almost diagnostic of cavernous haemangioma. A total extirpation of the lesion could be achieved using microsurgical techniques with relative ease.
The unusual location of the cavernoma in the interpeduncular cistern as well as trigeminal neuralgia as presenting feature make the case interesting.
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