1. Aggressive meningioma Robert M. Koffie Neurosurgery sub-intern July 19, 2012 Department of Neurosurgery Massachusetts General Hospital Harvard Medical School

2. Case Presentation HPI: 62 RHF 6 yrs ago p/w headaches, personality changes, & difficulty concentrating, and was found to have 7-cm parasagittal extra-axial enhancing lesion c/w meningioma. Multiple recurrence s/p surgery x4, several cycles of radiotherapy.

3. Oncological course: –2006: GTR, benign meningioma –2007: Recurrence, GTR, benign meningioma w/ atypical features Post-op fractionated radiotherapy –2009: Local recurrence, GTR, atypical meningioma –2010: Multifocal recurrence, proton SRS x3 –2011: Recurrence, GTR, atypical meningioma w/ anaplastic features –2012: Multifocal recurrence, ant. skull base invasion Case Presentation

4. T1 post gad: Multifocal parasagittal enhancing mass lesion with dura tail; another component centered in nasal cavity, filling ethmoid sinus, & bilateral orbits; contiguous anterior cranial fossa component Part 1 pre-op scans

5. Part 1 –Bifrontal craniotomy for resection of parasagittal tumor – 125 I brachytherapy seed placement Part 2 –Endonasal resection of tumor (ENT) –Bifrontal craniotomy for skull base tumor –Biorbital decompression (ENT/Oculoplastics) –Titanium + free flap skull base reconstruction (Plastics) Operative plan

6. T1 post gad: Enhancing mass lesion centered in nasal cavity, filling ethmoid sinus, & bilateral orbits; contiguous anterior cranial fossa component with dura tail, severe proptosis Non-contrast CT: Destruction of cribriform plate and surrounding bone, Brachytherapy seeds in place Part 1 post-op scans (Part 2 pre-op)

7. Intraoperative findings EndonasalSubfrontal

8. Post-op scans T1 post gad: s/p extensive cranial facial resection of tumor flap placement for skull base repair no residual tumor

9. WHO grades II and III meningioma Pathology

10. Design: Retrospective, 108 patients Goal: Define the long-term recurrence rate after GTR, role of radiation, factors predicting recurrence Results: Recurrence rates 7% (1 year), 41% (5 years), and 48% (10 years). Age, men, prominent nucleoli & high mitosis increases risk Recurrence rate (Atypical) Aghi MK et al, Neurosurgery, 2009

11. Recurrence rate (Atypical)

12. Design: Retrospective, 63 patients. Results: Survival after initial operation 82% (2y), 61%(5y) 40%(10y) +Survival benefit with repeat operation (53 vs 25 months) Surgery for recurrence Sughrue ME et al, J Neurosurg, 2010

13. Pathogenesis of Aggressive Meningioma BenignAtypicalAnaplastic Choy BA, Neurosurg Focus, 2011

14. Pathogenesis of Aggressive Meningioma BenignAtypicalAnaplastic Atypical Anaplastic

15. Krayenbühl N et al., Neurosurgery, 2007 WHO grade II WHO grade III De novo vs. transformed aggressive meningioma

16. Agar et al., Anal. Chem. 2010 Imaging Meningioma Progression: MALDI-MSI

17. POD8: Tension pneumocephalus s/p decompression POD10: Thrombosed free flap, CSF Leak s/p repair POD11: Lumbar drain POD15: Trach/PEG POD22: Doing well, transfer to rehab Hospital course

18. MGH Faculty – William T. Curry, M.D. – David N. Louis, M.D. MGH House staff – Gavin P. Dunn, M.D., Ph.D. – Peter E. Fecci, M.D., Ph.D. – Daniel A. Mordes, M.D., Ph.D Staff – Sylvia Weld – Neuro Nurses/NPs Acknowledgements

19. Thank you