1. ©2011 MFMER | slide-1 AANP 2011 CASE #10 Mark Jentoft MD Nancy Kois MD Bernd Scheithauer MD

2. Clinical History 11 year old ♂ 2 month history of HA 2 week history of confusion Parents thought he had the “flu”

3. Axial T1 with Contrast

4. Clinical History Gross/surgical –Firm/fibrous –Only basal portion near sphenoid wing reportedly dural attached –Surgical impression was that of a large meningioma –Gross total resection

5. H&E 100x

6. H&E 200x

8. H&E 400x

9. Differential Diagnosis?

10. S-100Col. IV CD 57MIB1

11. Additional stains/tests EMA, keratin, desmin, & myogenin –Negative RT-PCR for the synovial sarcoma SYT-SSX1 and SYT-SSX2 fusion transcripts –Negative Additional stains per report: –CD34---focal positivity –GFAP, Olig2---negative

12. Diagnosis Malignant peripheral nerve sheath tumor –Grade III (of IV)

13. Intracranial MPNST Rare! May occur in the setting of NF-1 Arise often in association with cranial nerves but may arise independent of them

14. Largest series to date 17 cases. –14♂ & 3♀ –All but one were adults Mean 39yo (5-69) –5 had NF-1 (29%) –1 with presumed NF-2 –4 with previous history of radiation –All were high grade lesions, grades III-IV

15. 8 arose from precursor lesions –2 from neurofibromas –2 from plexiform neurofibromas –4 from schwannomas Follow up available on 14 of the cases –12 died & 2 living have mult. recurrences –mean approx.1 year

16. Intracranial MPNST (Not associated with a Cranial Nerve) 18 Total cases in the latest published literature review. –12 adults 6 children –12♂ & 6♀ –4 had NF-1 (22%) Van den Munckhof P, Germans MR, Schouten-van Meeteren AY, Oldenburger F, Troost D, Vandertop WP.

17. –Possible origin Schwann and mesenchymal cells –Around perivascular nerve plexuses in the subarachnoid space –In adrenergic nerve fibers innervating cerebral arterioles –Meningeal branches of the trigeminal nerve Displaced neural crest cells into the developing central nervous system in embryonic life. Intracranial MPNST (Not associated with a Cranial Nerve) Van den Munckhof P, Germans MR, Schouten-van Meeteren AY, Oldenburger F, Troost D, Vandertop WP.

19. Follow Up 1 st surgery: Nov 2011 –Gross total resection of the tumor Subsequent workup (including PET): –No metastatic disease or other primary lesions Recurrence/2 nd surgery: Jan 2011 –3 areas of tumor identified and resected Temporalis muscle nodule (isolated from brain) Nodule in Anterior temporal fossa (isolated from brain) Thickening of arachnoid in the Sylvian fissure Subsequent chemotherapy and radiation

20. Follow up Patient is still alive at 7 months from initial presentation Recent biopsy proven involvement of the overlying skin on the right temple. No intracranial involvement at this time 1st surgery 2nd surgery Skin biopsy Nov. Dec. Jan. Feb. Mar. Apr. May. Jun. Jul Radiotherapy Chemotherapy Initiated

21. Questions?

22. References Scheithauer BW, Erdogan S, Rodriguez FJ, Burger PC, Woodruff JM, Kros JM, Gokden M, Spinner RJ: Malignant peripheral nerve sheath tumors of cranial nerves and intracranial contents a clinicopathologic study of 17 cases. Am J Surg Pathol 2009; 325-338. Van den Munckhof, Germans MR, Schouten van Meeteren AYN, Oldenburger F, Troost D, Vandertop PW:. Recurring intracranial malignant peripheral nerve sheath tumor: Case report and Systematic review of the literature. Neurosurgery 2011; 68:4