Primary intracranial leiomyosarcoma

Last revised by Dr Rohit Sharma on 22 Oct 2019

Primary intracranial leiomyosarcomas are primary malignant tumors derived from smooth muscle cell lineage in the cranial vault.

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.

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.

Intracranial leiomyosarcoma usually appears as an extra-axial mass 1,2, with intra-axial mass being a rare presentation 3,4.

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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Cases and figures

  • Case 1
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