1320 Acute Symptomatic Hemorrhage Associated With Peripheral Nerve Tumors in NF Patients

INTRODUCTION: Although vasculopathy is a known complication of neurofibromatosis type 1(NF1) and peripheral nerve tumors, significant spontaneous symptomatic hemorrhage is uncommon. The literature contains case reports of devastating tumor hemorrhage but we present our series of patients with large symptomatic hemorrhage associated with peripheral nerve tumors. METHODS: A retrospective review of patients from Penn State Health including age, sex, location of tumor/hemorrhage, treatment, prior and subsequent hemorrhage events and outcomes were recorded. RESULTS: Median age at time of hemorrhage was 41. One patient with NF2 suffered spontaneous hemorrhage of a sciatic schwannoma requiring urgent surgery for intractable pain. The remaining patients carried a diagnosis of NF1 and suffered symptomatic hemorrhages associated with plexiform neurofibromas(PNF) located in the trunk, glute, and lower extremities. One hemorrhage occurred 2 months after resection of a MPNST. One occurred after seizure resulting in a fall and dislocation of the hip contralateral to the hemorrhage. The remaining bleeds occurred after no trauma. Two or more occurred in all NF1 patients. Evidence of prominent vasculature in all and arteriovenous shunting in some was noted. Misdiagnosis was common leading to delayed or inappropriate treatment in some cases. Some underwent one or more endovascular treatments. Surgery was carried out in the NF2 and some NF1 patients. All patients survived their hemorrhages. CONCLUSIONS: Hemorrhage associated with peripheral nerve tumors is an uncommon but potentially debilitating complication. In patients with PNF, recurrent hemorrhage is frequent. Providers, patients and families should be educated regarding this issue in order to facilitate diagnosis and prevent delayed treatment.