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AJNR Awards, New Junior Editors, and more. Read the latest AJNR updates

Frontal Lobe Intraparenchymal Schwannoma

  • Background:
    • ​​Nerve sheath tumors, most common of which are schwannomas, account for 8% of primary brain tumors. They are benign in nature and mainly relate to cranial nerves; 90% involve the vestibular nerve.
    • Intraparenchymal schwannomas are very rare and though their origin remains uncertain, possible explanations include:
      • conversion of pial to Schwann cells
      • periarterial and perivascular nerve plexus Schwann cell existence
      • presence of similar myelinated cells in the tela choroidea
  • Clinical Presentation:
    • Is that of any other patient with a central nervous system mass: raised intracranial pressure, focal neurologic symptoms, and seizures
    • Unlike vestibular schwannomas, they are most prevalent in children and young adults.
  • Key Diagnostic Features:
    • Superficial or periventricular in location; well-demarcated and rounded or oval in shape 
    • Hypo- or isointense on T1WI, heterogeneous on T2WI (likely owing to calcification), and demonstrates homogenous contrast enhancement
    • Central necrotic or peripheral cystic areas are frequently seen, sometimes secondary to hemorrhage.
    • Calcification is more commonly seen compared with vestibular schwannoma, as is perilesional edema and gliosis.
    • Diffusion restriction is not present as in vestibular schwannoma.
  • Differential Diagnoses:
    • Lymphoma: often shows homogenous enhancement; however, would tend to restrict on DWI
    • Infiltrative astrocytoma: lesion tends to be more heterogeneous with variable enhancement depending on the tumor grade
    • Meningioangiomatosis: lesions often show minimal or no contrast enhancement and variable degree of calcification
  • Histology:
    • The morphology was typical of schwannoma, with mostly Antoni A pattern, formation of Verocay bodies, and S100-positivity.
    • The pattern was not that of meningioangiomatosis, in which there is perivascular proliferation of spindle cells, which are S100-negative.
    • The lack of GFAP and CD34 expression excludes a glioma and a solitary fibrous tumor, respectively.  
  • Treatment:
    • Surgical resection: the prognosis is very good following resection, with recurrence being rare.
    • Schwannomas are slow-growing tumors that involve the nerve sheath rather than the nerve itself, so they can often be resected separately.
August 17, 2017

A 54-year-old woman with right-sided neck pain and a history of epilepsy

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Print ISSN: 0195-6108 Online ISSN: 1936-959X

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