Intraspinal clear cell meningioma: Diagnosis and management: Report of two cases

OBJECTIVE AND IMPORTANCE: Intraspinal clear cell meningioma is a rare morphological variant of meningioma. Only 13 case reports are found in the literature; therefore, no management strategy has been defined for this tumor type. This article describes two patients, reviews the literature, and proposes a treatment plan for clear cell meningioma. CLINICAL PRESENTATION: Two female patients, 22 months and 8 years of age, respectively, presented with localized neck and leg pain that limited their ability to walk. Magnetic resonance imaging revealed intradural tumors, a cervical intramedullary neoplasm in the younger patient, and a cauda equina tumor in the older child. INTERVENTION: Both patients underwent radical resection of their intradural tumor. Both tumors, however, recurred shortly (5 and 6 mo) after the initial operation. During the second operation, a radical removal was performed on each patient. Both patients received adjuvant radiotherapy. In addition, the younger patient developed posterior fossa metastasis 20 months after intraspinal surgery. CONCLUSION: Intraspinal clear cell meningiomas are very uncommon tumors. The clinical course in our two patients supports the reported 40% recurrence rate within 15 months. These tumors also can disseminate within the central nervous system. We recommend serial imaging studies every 3 months. For recurrent tumors, we recommend localized radiation therapy after reoperation.