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UNC Neuroradiology-Neuropathology Conference

UNC Neuroradiology-Neuropathology Conference. November 20 12 Stephen Bagg, MD Fellow in Neuroradiology. Case 1- 57 y/o female with no sig PMHx 1 month hx of progressive Dizziness Diplopia Falls Physical Exam CN 6 palsy R facial droop and R tongue deviation L facial numbness

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UNC Neuroradiology-Neuropathology Conference

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  1. UNC Neuroradiology-Neuropathology Conference November 2012 Stephen Bagg, MD Fellow in Neuroradiology

  2. Case 1- 57 y/o female with no sig PMHx • 1 month hx of progressive • Dizziness • Diplopia • Falls • Physical Exam • CN 6 palsy • R facial droop and R tongue deviation • L facial numbness • R sided dysmetria • Neurology localized abnormality to • Medulla, pons, cerebellum

  3. Brainstem Gliomas in Adults • In a series of 48 adults with a brainstem glioma by clinical and radiologic findings, most of the tumors fit into one of three categories: • 50% of patients had diffuse intrinsic low-grade gliomas(WHO I or II). Ppatientswere typically young adults with a long clinical history and no tumor enhancement. Patients treated by radiotherapy and had a 7.3 year median survival. Anaplastic change (as determined by MRI) developed in 50% of and was the most common cause of death. • 33% patients had malignant gliomas (WHO grade III or IV). Patients were typically older adults, presented with a short clinical history, and had enhancement and signs of necrosis. Patients were refractory to therapy and had a 11.2 month median survival. • 10% of patients had focal tectalgliomas. These were young and often presented with hydrocephalus. These tumors had an indolent course. Guillamo J-S, et al. Brain 124:2528-2539, 2001.

  4. Case 2- 21 month old male • Previously healthy • Recently noted by PCP to have macrocephaly • Developed Ataxia and N/V

  5. Atypical teratoidrhabdoid tumor (ATRT) • ATRTs are rare tumors that comprise approximately 1-2% of all pediatric brain tumors; however, in patients less than 3 years of age this tumor accounts for up to 20% of cases. • ATRT usually occurs in posterior fossa or supratentorial location and are only rarely in spinal region • ATRT is characterized by loss of the long arm of chromosome 22, which results in loss of the hSNF5/INI-1 gene and loss of INI-1 expression. • INI1, a member of the SWI/SNF chromatin remodeling complex, is important in maintenance of the mitotic spindle and cell-cycle control. • WHO grade IV. Overall survival in ATRT is poor with median survival of around 17 months. Ginn KF, Gajjar A. Front Oncol. 2012;2:114. Epub 2012 Sep 12.

  6. Imaging Features, ATRT • Heterogeneous mass/enhancement • Commonly contains cysts & hemorrhage • Leptomeningeal enhancement is common • Restricted Diffusion is common • Imaging Diff Dx = medulloblastoma • ATRT are generally more heterogeneous than medulloblastoma • Medulloblastomais more commmon

  7. Common neoplasms associated with intracranial tumoral hemorrhage YuguangL, et al. Journal of Clinical Neuroscience(2002); 9(6),637-639.

  8. Case 3- 36 y/o male • 1 year history of R hand and foot numbness • Otherwise healthy- • No significant past medial history

  9. Inhibin IHC

  10. Hemangioblastoma • Hemangioblastomais a benign CNS neoplasm (WHO grade I), representing 1.5%–2.5% of all intracranial tumors and 7%–12% of posterior fossa tumors. It most commonly arises in the cerebellum but may also arise in the cerebral hemispheres, medulla, and spinal cord. Peak incidence is 3rd to 6th decades. • 75% of hemangioblastomas are associated with sporadic mutations involving the Von Hippel-Lindau (vHL) gene located at the short arm of chromosome 3, whereas the remaining 25% occur in the setting of the Von Hippel-Lindau syndrome. This is an autosomal dominant syndrome caused by a germlinemutation in the vHL tumor suppressor gene. • Individuals with the VHL syndrome are at risk of developing renal cell carcinoma and hemangioblastomain addition to pancreatic and renal cysts, cystadenomasof the epididymis, and endolymphaticsac tumors. • The stromal cells stain for Inhibin A, which is a glycoprotein normally secreted by ovarian granulosacells and testicular Sertoli and Leydig cells to inhibit pituitary follicle-stimulating hormone. Carney EM, et al. Am J SurgPathol2011;35:262–267.

  11. Imaging Features, Hemangioblastoma • Cystic mass with enhancing mural nodule • Associated flow voids • Thoracic > Cervical > Lumbar • Evaluate posterior fossa for additional lesions • Differential diagnosis: Astrocytoma, Ependymoma, AVF/AVM

  12. Case 4- 58 y/o F • Presented to outside institutionwith altered mental status • Possible complaint of blurry vision • PMHx • Diabetes, Hypertension

  13. ASL CBF map

  14. Meningioma

  15. Thank you

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