Transcript AANP 2011 CASE #10 - University of Pittsburgh

AANP 2011
CASE #10
Mark Jentoft MD
Nancy Kois MD
Bernd Scheithauer MD
©2011 MFMER | slide-1
Clinical History
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11 year old ♂
2 month history of HA
2 week history of confusion
Parents thought he had the “flu”
Axial T1 with Contrast
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
H&E 100x
H&E 200x
H&E 200x
H&E 400x
H&E 400x
Differential Diagnosis?
S-100
Col. IV
CD 57
MIB1
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
Diagnosis
• Malignant peripheral nerve sheath tumor
– Grade III (of IV)
Intracranial MPNST
• Rare!
• May occur in the setting of NF-1
• Arise often in association with cranial
nerves but may arise independent of them
• 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
• 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
Van den Munckhof P, Germans MR, Schouten-van Meeteren AY,
Oldenburger F, Troost D, Vandertop WP.
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.
Intracranial MPNST (Not associated with a Cranial Nerve)
– 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.
Follow Up
• 1st surgery: Nov 2011
– Gross total resection of the tumor
• Subsequent workup (including PET):
– No metastatic disease or other primary lesions
• Recurrence/2nd 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
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
2nd surgery
1st surgery
Chemotherapy
Initiated
Skin biopsy
Radiotherapy
Nov.
Dec.
Jan.
Feb.
Mar.
Apr.
May.
Jun.
Jul
Questions?
References
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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.
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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