Clinical and radiological aspects of cerebellopontine neurinoma presenting with recurrent spontaneous bleedings

Abstract

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.

Clinically significant intratumoral hemorrhage in patients with vestibular schwannoma

“…To date, fewer than 50 cases of hemorrhagic VS have been reported in the English literature . In 2014, Niknafs et al performed a review of the literature and identified 39 cases from 18 published articles .…”

“…The majority of cases of hemorrhagic VS require prompt surgical intervention because observation and radiosurgery do not alleviate mass effect and risk repeated hemorrhage . Prior to surgery, a focused history and laboratory testing should be obtained to evaluate for primary or acquired bleeding diathesis.…”

“…Subtotal resection may be required in unstable or frail patients in order to limit anesthetic time. The primary benefit of complete tumor removal is the reduced theoretical risk of catastrophic postoperative hemorrhage that may occur after STR, particularly if a large tumor remnant is left …”

“…We speculate that microhemorrhage provides the inciting spark; however, persistent bleeding generally requires a secondary factor that promotes greater arteriovenous pressure or impaired coagulation. Prior reports have outlined several potential secondary triggers, including hypertension, pregnancy, excessive straining (e.g., weight lifting), minor head trauma, and antiplatelet or anticoagulant therapy . The issue of head trauma is particularly relevant to the VS population since older age and disequilibrium may increase fall risk .…”

“…Also, there have been reports of likely de novo aneurysm formation in patients with VS treated with stereotactic radiosurgery . Additional reports of tumoral hemorrhage following prior subtotal resection have also been described . Third, given the retrospective nature of study, details regarding duration of anticoagulation use in the 33 patients was not known.…”