Child's Nervous System

, Volume 34, Issue 8, pp 1605–1608 | Cite as

Intracranial malignant peripheral nerve sheath tumor variant: an unusual neurovascular phenotype sarcoma case invading through the petrous bone

  • Oliver D. MrowczynskiEmail author
  • Robert J. Greiner
  • Malika Kapadia
  • Julie C. Fanburg-Smith
  • Mark R. Iantosca
  • Elias B. Rizk
Case Report



Intracranial malignant peripheral nerve sheath tumor (MPNST) is exceedingly rare. Previously reported cases of intracranial MPNST have been associated with development within a prominent cranial nerve.


This is the first report of an MPNST with both nerve sheath and vascular phenotype that follows the neurovascular bundle, without arising in a major cranial nerve or in the setting of neurofibromatosis type 1 (NF1).


The patient is a 14-year-old boy with a history of worsening headaches for the past several months, left-sided hearing loss, nausea, vomiting, and vertigo. MRI was performed that demonstrated a large extra-axial tumor compressing the left infratemporal posterior temporal region. The tumor was associated with significant destruction of the superior portion of the petrous bone and extension through the petrous into the upper posterior fossa, immediately below the tentorium. The patient underwent surgical debulking and adjuvant chemotherapy with doxorubicin and ifosfamide. Pathology demonstrated a variant malignant peripheral nerve sheath tumor with both nerve sheath and vascular phenotype by immunostains. The patient’s symptoms improved following treatment.


We present the first reported case of an intracranial MPNST variant that developed along the neurovascular bundle as a sarcoma with both nerve sheath and vascular phenotype through the petrous bone and not associated with a major cranial nerve or with stigmata of neurofibromatosis type 1 (NF1). Although this is an extremely unusual presentation due to location and lack of prominent cranial nerves in that location, it is not unusual for benign nerve sheath tumors to follow the neurovascular bundle through foramen of cortical long bone or pelvis. This case suggests that physicians should incorporate intracranial MPNST variant into their differential diagnosis in the cranium, even when tumor is not located near a prominent cranial nerve. Surgical debulking and adjuvant chemotherapy with doxorubicin and ifosfamide has led to improvement in patient symptoms.


Malignant peripheral nerve sheath tumor Vascular phenotype Intracranial Angiosarcoma Petrous bone 



We would like to acknowledge and sincerely thank Sara T. Langan and Dr. Charles S. Specht of the Neurosurgery and Pathology Departments for all of their efforts in the preparation of this article.

Compliance with ethical standards

Conflict of interest

On behalf of all authors, the corresponding author states that there is no conflict of interest.


  1. 1.
    Farid M, Demicco EG, Garcia R, Ahn L, Merola PR, Cioffi A, Maki RG (2014) Malignant peripheral nerve sheath tumors. Oncologist 19:193–201. CrossRefPubMedPubMedCentralGoogle Scholar
  2. 2.
    Widemann BC (2009) Current status of sporadic and neurofibromatosis type 1-associated malignant peripheral nerve sheath tumors. Curr Oncol Rep 11:322–328CrossRefPubMedGoogle Scholar
  3. 3.
    Voorhies J, Hattab EM, Cohen-Gadol AA (2013) Malignant peripheral nerve sheath tumor of the abducens nerve and a review of the literature. World Neurosurg 80:654.e1–654654.e8. doi:
  4. 4.
    Ziadi A, Saliba I (2010) Malignant peripheral nerve sheath tumor of intracranial nerve: a case series review. Auris Nasus Larynx 37:539–545. CrossRefPubMedGoogle Scholar
  5. 5.
    Akimoto J, Ito H, Kudo M (2000) Primary intracranial malignant schwannoma of trigeminal nerve. A case report with review of the literature. Acta Neurochir 142(5):591–595CrossRefPubMedGoogle Scholar
  6. 6.
    Lp K, Monica I, Uppin M, naidu KV (2014) Large oculomotor nerve schwannoma—rare entity: a case report with review of literature. J Cancer Res Ther 10:1098–1100. CrossRefGoogle Scholar
  7. 7.
    Nakayama Y, Watanabe M, Suzuki K et al (2012) Malignant peripheral nerve sheath tumor of the trigeminal nerve: Clinicopathologic features in a young adult patient. Neuropathology 33:541–546. PubMedCrossRefGoogle Scholar
  8. 8.
    McMenamin ME, Fletcher CD (2001) Expanding the spectrum of malignant change in schwannomas: epithelioid malignant change, epithelioid malignant peripheral nerve sheath tumor, and epithelioid angiosarcoma: a study of 17 cases. Am J Surg Pathol 25(1):13–25CrossRefPubMedGoogle Scholar
  9. 9.
    Kroep JR, Ouali M, Gelderblom H, le Cesne A, Dekker TJA, van Glabbeke M, Hogendoorn PCW, Hohenberger P (2011) First-line chemotherapy for malignant peripheral nerve sheath tumor (MPNST) versus other histological soft tissue sarcoma subtypes and as a prognostic factor for MPNST: an EORTC Soft Tissue and Bone Sarcoma Group study. Ann Oncol 22:207–214. CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  • Oliver D. Mrowczynski
    • 1
    Email author
  • Robert J. Greiner
    • 2
  • Malika Kapadia
    • 2
  • Julie C. Fanburg-Smith
    • 3
  • Mark R. Iantosca
    • 1
  • Elias B. Rizk
    • 1
  1. 1.Department of NeurosurgeryPennsylvania State University College of MedicineHersheyUSA
  2. 2.Department of Pediatric Hematology/OncologyPennsylvania State University College of MedicineHersheyUSA
  3. 3.Department of PathologyPennsylvania State University College of MedicineHersheyUSA

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