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
- 1. Brown HG, Burger PC, Olivi A, Sills AK, Barditch-Crovo PA, Lee RR. Intracranial leiomyosarcoma in a patient with AIDS. (1999) Neuroradiology. 41 (1): 35-9. Pubmed
- 2. Lerdlum S, Lalitanantpong S, Numkarunarunrote N, Chaowanapanja P, Suankratay C, Shuangshoti S. MR imaging of CNS leiomyosarcoma in AIDS patients. (2004) Journal of the Medical Association of Thailand = Chotmaihet thangphaet. 87 Suppl 2: S152-60. Pubmed
- 3. Eckhardt BP, Brandner S, Zollikofer CL, Wentz KU. Primary cerebral leiomyosarcoma in a child. (2004) Pediatric radiology. 34 (6): 495-8. doi:10.1007/s00247-003-1123-2 - Pubmed
- 4. Gautam S, Meena RK. Primary intracranial leiomyosarcoma presenting with massive peritumoral edema and mass effect: Case report and literature review. (2017) Surgical neurology international. 8: 278. doi:10.4103/sni.sni_219_17 - Pubmed