1 / 27

2012 Diagnostic Slide Session Case 6

Misti Coronel, MD David W. Andrews, MD Lawrence Kenyon, MD, Ph.D. Thomas Jefferson University, Philadelphia PA. 2012 Diagnostic Slide Session Case 6. Pt. history. 34yo F from Saudi Arabia with long history of a L frontal tumor 2002 resection  dx: pilocytic astrocytoma

kenton
Download Presentation

2012 Diagnostic Slide Session Case 6

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. Misti Coronel, MD David W. Andrews, MD Lawrence Kenyon, MD, Ph.D. Thomas Jefferson University, Philadelphia PA 2012 Diagnostic Slide SessionCase 6

  2. Pt. history • 34yo F from Saudi Arabia with long history of a L frontal tumor • 2002 resection  dx: pilocytic astrocytoma • interim radiation therapy • 2005 resection • 2009 resection • interim chemotherapy • 2010 resection • interim radiation therapy • presented to Thomas Jefferson University Hospital in 2012 for resection and further treatment

  3. Presenting symptoms • constant headache • worsening R-sided weakness • seizures • expressive aphasia

  4. MRI • previous L frontoparietal craniotomy site • adjacent large mass extending into operculum and frontal parenchyma • 7 mm midline shift • extensive patchy enhancement

  5. T2 FLAIR T1 post-gadolinium • Clear surgical plane intraoperatively • The tumor was partially resected

  6. Discussion

  7. H&E • large, pleomorphic tumor cells with prominent nucleoli • bizarre giant cells with multinucleation and granular eosinophilic inclusions • widespread necrosis • high mitotic rate • minimal cytoplasmic vacuolation • no lymphocytic inflammatory infiltrate

  8. Neurofilament M+H

  9. Neurofilament L

  10. Immunohistochemistry • Positive markers • Neurofilament M+H, neurofilament L • focal CD68 (possible autophagic activity) • Negative markers • S100 • GFAP • synaptophysin, chromogranin • Neu N • HMB45, MelA

  11. Case summary • longstanding brain tumor treated with multiple resections, chemotherapy, and radiation • initially diagnosed as pilocytic astrocytoma • sampling error? • slides not available for our review • subsequent specimens diagnosed as anaplastic ganglioglioma

  12. 2005 Resection 400X 200X 400X

  13. GFAP, 100X GFAP, 400X synaptophysin, 400X

  14. NF M+H, 400X NF-L, 400X NeuN, 400X

  15. Ganglioglioma • ganglioglioma—a rare tumor (0.5-1% of adult neuroepithelial tumors) composed of neoplastic ganglion cells and astrocytes • malignant change uncommonly occurs; when it does it is generally limited to the glial component • rare case reports of neuronal cell transformation to a neuroblastoma-like element • another case report describes anaplastic cells with both neuronal and astrocytic features

  16. Transformation of a ganglioglioma A. Tarnaris et al., 2006

  17. “Anaplastic gangliocytoma”—in a cow • large, pleomorphic tumor cells with multinucleation and prominent nucleoli • NSE+, NF+, very rare GFAP+ cells • some cells with cytoplasmic eosinophilic inclusions shown to be lysosomes on EM

  18. Electron microscopy The large eosinophilc inclusions were CD68 negative. EM- membrane bound electron dense inclusions. Misfolded proteins in ER?

  19. Significance • our tumor does not appear to have a glial component • only positive stains were for neuronal markers • previous reports of transformed gangliogliomas, but in these cases either the glial component was malignant or the neuronal component was more primitive (neuroblastoma-like) • a malignant, purely neuronal tumor such as ours does not appear to have been previously proven using modern immunohistochemical methods

  20. Follow up • patient received post-op radiation • remains in the hospital • disease has been stable • problems with wound dehiscence of the skull flap

  21. Follow up imaging T1 post-gadolinium T2 FLAIR

  22. References • David, K. M., S. Sanctis, P. D. Lewis, A. M. S. Noury, and J. M. R. Edwards. 2000. Neuroblastomatous recurrence of ganglioglioma. J Neurosurg 93:698-700. •  Jay, V., J. Squire, L. E. Becker, and R. Humphreys. 1994. Malignant transformation in a ganglioglioma with anaplastic neuronal and astrocytic components. Report of a case with flow cytometric and cytogenetic analysis. Cancer 73:2862-8. •  Kimura, K., Y. Wada, H. Kondo, Y. Ishikawa, and K. Kadota. 1999. Anaplastic gangliocytoma with eosinophilic cytoplasmic granules in a cow. J Vet Med Sci 61:983-5. •  Kernohan, J., J. R. Learmonth. 1932. Neuroblastomas and gangliocytomas of the central nervous system. Brain 55:287-310. •  Tarnaris, A., C. O'Brien, and R. M. Redfern. 2006. Ganglioglioma with anaplastic recurrence of the neuronal element following radiotherapy. Clin Neurol Neurosurg 108:761-7.

More Related