Primary intracranial leiomyosarcomas are primary malignant tumors derived from smooth muscle cell lineage in the cranial vault.
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Epidemiology
Primary intracranial leiomyosarcomas are extremely rare. Most commonly, these lesions have been described as EBV-associated smooth muscle tumors in patients who are immunocompromised due to HIV infection.
Clinical presentation
Patients can have an incredibly varied clinical presentation, including a history of headaches, visual changes and other focal neurological deficits, seizures, and/or enlarging palpable cranial mass/swelling 4.
Radiographic features
Intracranial leiomyosarcoma usually appears as an extra-axial mass 1,2, with intra-axial mass being a rare presentation 3,4.
MRI
The signal characteristics are non-specific 4.
- T1: isointense to hypointense
- T2/FLAIR: variable
- T1 C+ (Gd): vividly enhancing and heterogeneous
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