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

5. 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). Ppatients were 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 tectal gliomas. These were young and often presented with hydrocephalus. These tumors had an indolent course.
Guillamo J-S, et al. Brain 124: , 2001.

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

9. Atypical teratoid rhabdoid 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.

10. 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
Medulloblastoma is more commmon

11. Common neoplasms associated with intracranial tumoral hemorrhage
Yuguang L, et al. Journal of Clinical Neuroscience(2002); 9(6),
Common neoplasms associated with intracranial tumoral hemorrhage

12. 1 year history of R hand and foot numbness Otherwise healthy-
Case y/o male
1 year history of R hand and foot numbness
Otherwise healthy-
No significant past medial history

14. Inhibin IHC

15. Carney EM, et al. Am J Surg Pathol 2011;35:262–267.
Hemangioblastoma
Hemangioblastoma is 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 germline mutation in the vHL tumor suppressor gene.
Individuals with the VHL syndrome are at risk of developing renal cell carcinoma and hemangioblastoma in addition to pancreatic and renal cysts, cystadenomas of the epididymis, and endolymphatic sac tumors.
The stromal cells stain for Inhibin A, which is a glycoprotein normally secreted by ovarian granulosa cells and testicular Sertoli and Leydig cells to inhibit pituitary follicle-stimulating hormone.
Carney EM, et al. Am J Surg Pathol 2011;35:262–267.

16. 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

17. Presented to outside institution with altered mental status
Case y/o F
Presented to outside institution with altered mental status
Possible complaint of blurry vision
PMHx
Diabetes, Hypertension

18. ASL CBF map

19. Meningioma

20. Thank you