Glioblastoma in Beckwith-Wiedemann syndrome: first case report and review of potential pathomechanisms.

Beckwith-Wiedemann syndrome (BWS) is a rare congenital overgrowth syndrome associated with certain childhood tumours. We present the case of a 36-year-old lady with BWS who developed a left frontoinsular secondary glioblastoma. This is the first case report in the literature of glioblastoma associated with BWS. We explore similarities in the molecular pathomechanisms of BWS and glioblastoma.

Multiple Meningiomata in Cowden Syndrome.

Cowden syndrome (CS) is a rare, congenital disease with associated cancers, but in a neurosurgical context is typically considered part of Lhermitte-Duclos. This misrepresentation is the likely cause of under-diagnosis. Furthermore, the presence of meningiomata has been described in CS patients but its absence as part of the condition's major criteria suggest the correlation requires greater documentation. A 41-year-old woman with multiple cancers and a familial circumambience of CS was reviewed in clinic where multiple meningiomata were incidentally identified on MRI. Despite a lack of neurological impairment, and the general reluctance in treating the meningiomas of congenital disease with radiotherapy (RT), the patient underwent stereotactic radio-surgery (SRS) and at one-year post-procedure has reported no side effects or toxicity.