Hemorrhagic Vestibular Schwannomas: A Review of 6 Cases

Background/Objective Hemorrhagic vestibular schwannomas (HVS) are a rare clinical entity, with less than 50 reported cases in the English literature. The pathogenesis of acute intratumoral hemorrhage remains unknown since the majority of published cases have occurred in patients without precipitating trauma or underlying risk factors such as anticoagulation. Presentation is variable, however most patients experience acute decline in symptoms presumably caused by abrupt tumor enlargement from intratumoral bleeding. The current study aims to review our experience managing 6 patients with HVS that presented over the past 15 years to further define risk factors, patient course, and outcome.

Design: Retrospective chart review of all patients diagnosed with HVS, evaluated at a single tertiary acadmeic referall center between 2000–2015.

Outcome Measures Disease presentation, radiologic features, management strategy, and clinical outcome.

Results: Six HVS were evaluated between 2000 and 2015, representing ~0.3% of all vestibular schwannoma evaluated at the authors’ center during this time. The median age at time of diagnosis was 67 years (range 39–83), 5 of 6 cases were men, and all had sporadic unilateral tumors. No patients reported prior head trauma, however 3 of 6 were receiving warfarin for indications including atrial fibrillation and pulmonary embolism; the median INR in this subgroup was 2.3 (1.7–2.8). The most common presenting symptoms were hearing loss (AAO-HNS Class D), disequilibrium, and headache. Four patients reported recent vertigo, 3 had trigeminal hypesthesia, 2 acute facial paresis (HB 4), and 2 hydrocephalus. The median tumor size at diagnosis was 2.6cm (range 0.4–3.5) and 2 demonstrated macrocystic features. Four of 6 (67%) HVS had focal areas of pre-contrast T1-weighted hyperintensity within the tumor, and 2 of 6 exhibited subarachnoid blood products. Five of 6 patients underwent microsurgical resection while one subject with an intracanalicular tumor was observed. The latter patient presented with sudden sensorineural hearing loss and acute vestibulopathy; however shortly after evaluation his dizziness and hearing loss spontaneously improved (Class D to B) and there has been no evidence of growth for 68 months. Of the 5 subjects that underwent surgical treatment, 3 received gross total resection while 2 were managed with aggressive subtotal removal. The median HB score for patients with greater than 12 months of postoperative follow-up was 2. One of the two patients with preoperative HB grade 4 paresis has recovered good function (HB 2) over the course of 25 months following surgery, the second recently underwent surgery and follow-up data are not yet available. There were no perioperative deaths, and at a median follow-up of 19 months (0–69 months) no patient has experienced recurrence.

Conclusions: HVS are rare and commonly present with acute worsening of headache, hearing loss, imbalance, and cranial neuropathy. Contrasting previous reports, we found a high percentage (50%) of HVS associated with anticoagulation use. In surgically fit patients with large tumors, we advocate urgent microsurgical resection. Observation with serial radiography may risk repeated hemorrhage with further neurologic decline. The role of radiosurgery is not yet defined.