Presentation on theme: "Ray Peeples, MD. Case 1 50 y/o F with NF1 hx of meningioma debulking (2/10) and cervical neurofibroma removal (7/09) MRI studies showed an enhancing."— Presentation transcript:
Case 1 50 y/o F with NF1 hx of meningioma debulking (2/10) and cervical neurofibroma removal (7/09) MRI studies showed an enhancing superior cerebellar lesion growing over time and eventually causing obstructive hydrocephalus, the lesion was biopsied
In one series of 100 NF1 patients with biopsied tumors, there were… 50 pilocytic astrocytomas (PA) 28 diffuse astrocytomas (Grades II—5%, III—15%, IV—7%) 17 low- grade astrocytomas, subtype indeterminate 2 pilomyxoid astrocytomas 1 desmoplastic infantile ganglioglioma 1 conventional ganglioglioma 24 tumors arose in the optic pathways (14 PAs, 4 low-grade astrocytomas of indeterminate type, 4 anaplastic astrocytomas, 1 pilomyxoid astrocytoma, and 1 ganglioglioma). Most tumors arising in setting of NF1 are pilocytic astrocytomas (PAs) and, unlike their sporadic counterparts, have a distinctive predilection to involve the optic nerve, chiasm, and hypothalamus. NF1-related optic gliomas are typically in young children and afflict 15% to 20% of NF1 patients. NF-1 related optic gliomas seem to have a more indolent behavior than their sporadic counterparts and may even regress without treatment. Rodriguez FJ, et al. J Neuropathol Exp Neurol. 67:240–249, 2008.
Case 2 26 y/o M with 2 yr hx of back pain and lower extremity paresthesias MRI showed and intradural lesion in the L spine MRI brain showed multiple enhancing cerebellar lesions Spinal lesion was resected
Case 3 35 y/o M with 1 year hx of back pain and RLE weakness sent from outside institution with presumptive diagnosis of L3 schwannoma with MRI performed without contrast EMG showed L3 and S1 radiculopathy L3 mass was resected
Spinal syndromes are reported at clinical presentation in 6% to 8% of patients with neurosarcoidosis Spinal sarcoidosis can be intramedullary, intradural extramedullary, epidural, or in vertebral bodies. Most cases are intramedullary. Intradural, extramedullary spinal sarcoidosis is extremely rare, with only 8 cases reported in the literature as of 2006. Schaller B, et al. The Spine Journal 6:204–210, 2006.
Case 4 26 y/o M who presented in 10/2012 with seizure CT/MRI showed L frontal low density lesion in white matter with no enhancement, this lesion was biopsied