Multiple Meningiomata in Cowden Syndrome

Abstract

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.