Background: Primary intracranial leiomyoma is a rare smooth muscle tumor often associated with Epstein-Barr virus (EBV), with <30 cases reported worldwide. These tumors commonly occur in patients with immunocompromised status, especially those with human immunodeficiency virus. In the present report, we have described the case of an EBV-associated leiomyoma at the cerebellopontine angle. The patient had presented with trigeminal neuralgia, which, to the best of our knowledge, is the first reported anatomical location and presentation for this tumor type.
Case description: A 41-year-old male patient had presented with right-sided facial pain in the V1 and V2 dermatomes and previous workup and imaging studies. The patient had undergone treatment of a presumed right-side cerebellopontine angle meningioma as determined by the magnetic resonance imaging characteristics (no biopsy). The patient subsequently underwent right-sided retrosigmoid craniotomy and gross total resection of the tumor. The postoperative period was uneventful with resolution of the trigeminal neuralgia. Histopathologic examination revealed spindle cell neoplasm with histopathologic and immunohistochemical features consistent with leiomyoma. The tumor cells were positive for smooth muscle actin and desmin and were negative for S100, SOX-10, epithelial membrane antigen, glial fibrillary acidic protein, progesterone receptor, CD31, CD34, and E-cadherin.
Conclusions: Primary intracranial leiomyomas are rare tumors associated with EBV infection that occur in immunocompromised patients. These lesions should be considered in the differential diagnosis for patients with known immunocompromised status (e.g., human immunodeficiency virus), and tissue biopsy should be considered.
Keywords: Cerebellopontine angle; EBV; HIV; Leiomyoma; Trigeminal neuralgia.
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