- Department of Neurosurgery, Policlinic University of Messina, 98126 Messina, Italy
- Department of Neurosurgery, Hospital “Ospedali Riuniti”, 34149 Trieste, Italy
Department of Neurosurgery, Policlinic University of Messina, 98126 Messina, Italy
DOI:10.4103/2152-7806.112605Copyright: © 2013 Giuseppe M This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
How to cite this article: Giuseppe M, Mario G, Nicola N, Mauro C. Clinical and radiological aspects of cerebellopontine neurinoma presenting with recurrent spontaneous bleedings. Surg Neurol Int 28-May-2013;4:67
How to cite this URL: Giuseppe M, Mario G, Nicola N, Mauro C. Clinical and radiological aspects of cerebellopontine neurinoma presenting with recurrent spontaneous bleedings. Surg Neurol Int 28-May-2013;4:67. Available from: http://sni.wpengine.com/surgicalint_articles/clinical-and-radiological-aspects-of-cerebellopontine-neurinoma-presenting-with-recurrent-spontaneous-bleedings/
Background:Neurinomas are benign, usually encapsulated, tumors growing in peripheral nerve sheath with a high incidence in the cerebellopontine angle.
Case Description:We report a case of vestibular neurinoma (VN) with a “biphasic” pattern of intratumoral hemorrhage presenting with cephalalgia along with progressive ipsilateral mild impairment of both VII and VIII cranial nerves. A thorough preoperative magnetic resonance imaging study better characterized the patchy pattern of the round shaped lesion, resulting in three different intensity signals, due to the peculiar characteristics of the tumoral mass and the recurrent bleedings, respectively. Postoperatively, histological examination confirmed the diagnosis of neurinoma.
Conclusion:Hemorrhagic VN are rare tumors; from the first case described in 1974 only 43 more have been reported in the literature so far. Noteworthy, “biphasic” bleedings are even rarer. From an accurate review of the literature we collected and thus emphasized the radiological and clinical features of this rare entity. Eventually, we suggest that the early surgical removal of clots and tumor is essential to provide the best chance of neurological improvement.
Keywords: Biphasic bleeding, cerebellopontine angle, intratumoral hemorrhage, patchy pattern, vestibular Schwannoma
Neurinomas are benign, usually encapsulated, tumors growing in peripheral nerve sheath with high incidence in the cerebellopontine angle (CPA). As most of benign tumors, their presence is suspected by the onset of neurological signs in a slow-growing fashion caused by vascular or nervous conflicts.[
We report a case of vestibular neurinoma (VN) associated with a radiological “biphasic” pattern of intratumoral hemorrhage (ITH). A 65-year-old woman came to our attention complaining of sudden headache and progressive oral rhyme deviation. Neurological examination showed mild ipsilateral VII cranial nerve palsy and hearing loss. A computed tomography (CT) scan showed a 3 cm hyperdense mass occupying the CPA cistern with compression on adjacent cerebellar parenchyma [
The patient underwent surgical removal of the lesion performed by a suboccipital approach. Macroscopically, the tumor appeared as a soft, grayish mass surrounded by multiple clots. Histological examination confirmed diagnosis of VN type Antoni A. The patient's neurological status improved gradually with mild persistence of VII and VIII cranial nerve deficits.
Hemorrhagic CPA mass lesions have been rarely reported in literature,[
At time of presentation, patients are on average aged in their fifties (range from 15 to 71 years) with a slight male predominance (M/F: 1/3). The most frequently reported clinical symptoms are: Headache (27%), hearing disturbance (25.8%), cerebellar symptoms (16.9%), nausea, and vomiting (13.5%) [
Hemorrhagic VN presents with two basic kind of bleeding: Intratumoral and subarachnoid ones. The first form accounting for the 86.4% of cases (38 out of 44) consists in a hemorrhage inside the substance of the tumor itself.[
Generally speaking, neurinomas present a characteristic MRI signal pattern consisting in a slight hypointensity or isointensity on T1-weighted image and an increased signal in T2-weighted images. Whenever an intratumoral bleeding occurs, a decreased signal can be noted in correspondence of the center of the acute hematoma, due to the T2 shortening effects of deoxyhemoglobin. In subacute hematomas instead a peripheral hyperintensity extending inward is evident in both T1-weighted and T2-weighted images, explained by a T1 shortening effects of proton electron dipole–dipole secondary to methemoglobin interactions. Finally, in chronic hemorrhages a peripheral hypointensity in noted, and hemosiderin laden macrophages are considered responsible of this pattern.[
Among the cases described in the literature, most of them showed at the time of histological examination a mixed Antoni A/B pattern (18 out of 24, 75%), whereas only a few exhibited Antoni A pattern, suggesting that the mixed type are the most at risk of bleeding. A recent paper on a cohort of 67 VN patients[
The case herein described shows a radiologically evident biphasic hemorrhage that could confirm the hypothesis of recurrent bleedings along tumoral growth. Subsequent hemorrhages within the tumor mass seem to be quite rare and only four cases are reported in literature.[
In our case the second bleeding coincided with a sudden cephalalgia in a clinical history of progressive mild impairment of both VII and VIII cranial nerves. From a review of the literature, headache results as a predominant symptoms (27%, as shown in
In contrast, since a subtle clinical presentation is often the only finding in patients with hemorrhagic VN,[
Hemorrhagic VN surely represent a very rare entity. The literature review herein presented highlighted the following risk factors for tumoral bleeding: large size, mixed Antoni type, and secondary vascular changes, like increased/dilated vessels and vascular architecture resembling telangectasia.
The present case and the few similar ones described in literature suggest that subsequent bleedings of VN are associated with a higher morbidity rate. A radiological evidence of hemorrhagic neurinoma should therefore prompt a rapid surgical intervention, even in case of mild presenting symptoms, since only early removal of clots and tumor may provide the best chances of neurological improvement.
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