1 / 20

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

daria
Download Presentation

UNC Neuroradiology-Neuropathology Conference

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  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

More Related