Primary intracranial leiomyosarcoma: report of a case and review of the literature

Abstract

A 26-year old man presented with a 3-month history of a progressively enlarging palpable parieto-occipital mass. A CT scan indicated the lesion arose from the dura with bony destruction. A stealth assisted craniotomy was performed with the provisional diagnosis of osteoblastic meningioma. Further histopathologic analysis of the intracranial mass was consistent with leiomyosarcoma. Staging evaluation, including CT and PET scans, demonstrated no other sites of disease. Despite complete surgical resection and radiotherapy to the resection site, the disease recurred locally and systematically 5 months later. Primary intracranial mesenchymal tumors are rare and few cases have been previously reported. Outcomes have been universally poor and current therapeutic approaches appear to have only limited benefit.

Figure 1

CT scan of the head shows a large vascular soft tissue mass involving the meninges and invasion of right parietal bone.

Figure 2

(a) Malignant spindle cells, pleomorphism, and high nucleocytoplasmic ratio. (b) Immunostain positive for smooth muscle actin.