Angioleiomyoma, a rare intracranial tumor: 3 case report and a literature review

Three cases of intracranial angioleiomyoma (ALM) in our neurosurgery center are reported in detail. ALM is a benign soft tissue tumor comprised of mature smooth muscle cells and a prominent vascular component, which is extremely rare as a primary intracranial lesion. Altogether, only 12 cases were recorded in the literature to date, to the best of our knowledge. Case 1 is the second report of intra-sella ALM, a 51-year-old woman presented with visual deterioration for 2 months. An MRI showed an intra-sella 3-cm tumor, partially flame-like, enhanced with gadolinium. Using microscopic endonasal transsphenoidal approach, the tumor was completely resected with great difficulty. At 11 days post-surgery, she died of a sudden catastrophic nasal hemorrhage. An angiography revealed a pseudo-aneurysm of ICA (internal carotid artery). Case 2 is a 49-year-old man who presented with weakness of the lower limbs for 1 year. A large subtentorial mass was found affixed to the torcular and straight sinus, which was partially flame-like, dramatically enhanced as in case 1. Case 3 is that of a 77-year-old man. An ALM mass was revealed in the diploe of left temporal bone, and had eroded the inner table. Immunohistochemical workup confirmed the diagnosis of angioleiomyoma in all 3 cases. The radiology, operation, and complication of surgery in each case were discussed. In conclusion, intracranial ALMs are extremely rare, usually located ex-neuro axis (such as in our cases), in the sella, in posterior fossa, or in the skull. Magnetic resonance imaging (MRI) revealed a special feature of flame-like partial enhancement that may be helpful to distinguish ALM from pituitary tumors or meningiomas, and should result in the consideration of this rare tumor entity early on in the diagnostic process. A definitive diagnosis depends on histological analyses. The resection of ALM in certain locations is difficult and risky because of the rich blood supply.