MS-11 * CLEAR CELL PETRO-CLIVAL MENINGIOMA IN A FIVE YEAR OLD CHILD - HISTOPATHOLOGICAL DIAGNOSIS AND LONG-TERM CLINICAL FOLLOW-UP

Abstract

Intracranial meningiomas are rare in children. We report on a 5 year-old girl who was presented with a one-week history of cephalgia, ataxia, and left sided torticollis. Magnetic resonance imaging (MRI) revealed a dumbbell-shaped homogeneously petro-clival gadolinium-enhancing mass. A staged operative approach was chosen and a complete removal of the tumor was achieved. Due to recurrent tumor progressions, the child underwent several surgeries and two cranial radiations. None of the treatments were efficient enough to stop tumor progression. Consequently, the child died at the age of 14 after further extensive intra- and extracranial tumor progression. The initial histological examinations (at the age of 5 years) revealed a clear cell meningioma WHO grade II with an MIB-1 labeling index <1%, which gradually increased with every recurrence up to 10% by the last progression at the age of 13. Analogically, an increasing over-expression of EGF-, PDGF-, and VEGF-receptors with each recurrence was observed. In reviewing the literature, there have been only 10 reported cases of intracranial clear cell meningioma in children, none of which obtained a serial pathological investigation. The pathologic findings, treatment options, and present understanding of this tumor are discussed in this work.