Intracranial malignant peripheral nerve sheath tumor variant: an unusual neurovascular phenotype sarcoma case invading through the petrous bone
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.
KeywordsMalignant 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.
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Conflict of interest
On behalf of all authors, the corresponding author states that there is no conflict of interest.
- 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: https://doi.org/10.1016/j.wneu.2012.08.007
- 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. https://doi.org/10.1093/annonc/mdq338 CrossRefPubMedGoogle Scholar