Intracranial clear cell meningioma in two children with blood relations: two case reports and literature review

Intracranial clear cell meningioma (CCM) is very rare and often has an aggressive clinical course. The predilection site of intracranial CCM in children was cerebellopontine angle where represents challenging tumor resection because of the vicinity of brainstem, vertebral artery, and lower cranial nerve. Therefore, special consideration is required for this tumor. We report two cases with intracranial CCM in a family and reviewed the literature concerning pediatric intracranial CCM. Case 1 is a 4-year-old boy with a tumor at the right posterior fossa. Case 2 is an 8-year-old boy with a left basilar region tumor. Gross total resection and subtotal resection was achieved in case 1 and case 2, respectively. Case 1 had no tumor recurrence at 12 months after the operation. Case 2 received cyber-knife radiosurgery (CKS) after subtotal resection at 4 months, and the residual tumor had gradually shrunk. Comprehensive chromosomal number aberrations in both patients were revealed by array-comparative genomic hybridization, and loss of neurofibromatosis 2 (NF2) gene was the common genetic abnormality in the two patients. To the best of our knowledge, this is the first report concerned two patients with CCM in a family, and the findings in this article suggest that CKS is a safe and effective adjuvant therapy for residual CCM after operation and NF2 gene mutation plays a role in tumorigenesis of pediatric intracranial CCM.