Primary intracranial angioleiomyomas: diagnosis, treatment, and literature review

Intracranial angioleiomyomas (ALMs) are extremely rare, benign, mesenchymal soft tissue tumours. Early diagnosis is difficult without histopathology. We present 2 new cases of extra-axial ALMs involving the cavernous sinus and right temporal lobe. Moreover, present a literature review based on a search in PubMed using the words “angioleiomyomas, vascular leiomyomas, or angiomyomas”. This review exclusively included studies in English of primary intracranial ALMs with detailed clinical data. The histopathology of the presented cases was similar. Both lesions consisted of thick-walled vessels blending with smooth muscle cells and bands of collagen in a disorganized pattern. Mitosis, cytological atypia, necrosis, or pleomorphism was rarely observed. The Ki-67 labelling index was less than 1 %. Smooth muscle actin, desmin, and vimentin were positive, but cytokeratin and HMB45 were negative. Multifocality of mature fat cells was observed in one case. To date, only 12 cases of intracranial ALMs have been described. The mean age of these patients is 45.7 years, and they are predominantly male (M/F ratio 4.5:1). Neuroradiologic characteristics were indistinctive and were all misdiagnosed preoperatively. Intracranial ALMs are rare independent entities and favourable outcomes can be achieved by complete resection. Though rare, the possibility of ALM is considered during preoperative assessment.