gms | German Medical Science

Objective: Angioleiomyoma, often found in subcutaneous tissue, are known to be benign vascular smooth muscle tumors. Reports on their intracranial location are, however, very scarce. Nevertheless, there might be a need for neurosurgical resection of those rare tumors in selected cases. We, therefore, aimed to assess clinical, radiological, and pathological features of intracranial angioleiomyoma.

Methods: We retrospectively assessed all consecutive patients with neurosurgically treated intracranial angioleiomyoma, confirmed by neuropathological analysis, at a single neurosurgical institution between 2013 and 2021. Clinical and imaging data were collected, and histological tissue sections were reanalyzed. In addition, a corresponding literature review was performed using the PubMed library.

Results: Overall, four female and three male patients with a median age of 45 years (range: 33-76) were identified. An infratentorial lesion was found in three cases (42.9%). Other tumor locations were the cerebral falx, the parietoocipital and frontal lobes and the cavernous sinus. Three patients presented specific symptoms correlating with tumor location while the others suffered from unspecific headaches or dizziness. In MR-imaging, all lesions were hypo- to isointense on T1-weigthed and hyperintense on T2-weighted images. Gadolinum enhancement was seen in all cases. All lesions were completely removed, and no perioperative complications occurred. In neuropathological assessment, all tumors were immunohistochemically positive for SMA (smooth muscle actin). The Ki-67 labeling index was ≤ 3%. All patients were discharged after four to eight days postoperatively without new permanent deficits. At a median follow-up time of 6 months (range: 3 - 41 months), none of the patients presented recurrence. In the literature, 41 additional cases of intracranial angioleiomyomas were identified.

Conclusion: In general, intracranial angioleiomyoma seem to show a benign behavior and can successfully be treated with gross total resection. A well differentiated SMA positive smooth muscle component arranged around the vessels usually leads to the neuropathological diagnosis. With other entities such as meningeomas or vestibular schwannomas often being the primary differential diagnosis, knowing the benign course of this rare pathology is important for patient counseling.