Primary familial brain calcification (PFBC) is an idiopathic pathology characterized by the development of calcific deposits in the supratentorial region such as telencephalon and diencephalon but also, in more extensive forms, in the cerebellum. Meningiomas are among the most common central nervous system (CNS) tumors generally related to a good prognosis. The simultaneous presence of intracerebral or extra-axial tumors and PFBC represents an exceedingly rare occurrence. A 72-year-old female patient was admitted to our department because of anoculogyric crisis followed by generalized seizures. She performed a brain CT scan which showed widespread hyperdensities in the paraventricular supratentorial region, basal ganglia and at the level of bilateral cerebellar dentate nuclei, characteristics of PFBC. Concomitant left frontal and smaller right temporal extra-axial lesions were identified and then confirmed in a brain MRI. The patient underwent a microsurgical resection of the lesion and subsequent histological examination reported a meningothelial meningioma (WHO Grade I). According to our literature review, this is the first paper that reports the coexistence of both intracranial meningiomas and PFBC. To date, it is not possible to provide an exact correlation between pathogenesis and genetic mechanism underlying this association.
Keywords: Brain tumors; CNS, Central nervous system; Craniotomy; Gamma knife; Meningiomas; PFBC, Primary familial brain calcification; Primary familial brain calcification; SSS, Superior sagittal sinus.
© 2023 The Authors. Published by Elsevier Inc. on behalf of University of Washington.