Paediatric clear cell meningioma with multiple distant recurrences after presumed intra-operative cell spread

Abstract

Meningiomas are common neoplasms accounting for 24– 30% of all primary intracranial tumours but are rarely seen during childhood [1]. If there are no signs of atypia, brain invasion or mitotic activity, most tumours are classified as WHO grade I tumours, depending on their histological subtype. However, there are histological variants with known aggressive course such as rhabdoid and papillary meningiomas (WHO grade III) and chordoid and clear cell variants (WHO grade II). Of these, clear cell meningiomas (CCMs) account for 0.2% of all meningiomas, thus being the rarest variant [2]. They tend to affect both children and young adults (mean age 29 years, range 2–82 years) [2, 3]. CCMs occur most frequently in the cerebellopontine angle and cauda equina [1], often show recurrences, occasionally cerebrospinal fluid (CSF) seeding and are generally associated with a more aggressive behaviour [2]. The risk to develop a higher grade tumour is twice as high for males than for females [4]. Tumours with a non-skull base location more likely have a WHO grade II or III than skull-based tumours [4]. Because of their rarity, large single institution series of CCMs are rare. Most of the clinical data regarding behaviour of these uncommon tumours is based on only two larger studies, one from India with nine cases and one American study with 13 cases, with two and eight recurrences, respectively [2, 5]. At our institution, the presented case is the second tumour since its introduction in the WHO classification in 2000.