Primary Meningeal Sarcomas in Two Children

Abstract

Primary meningeal sarcomas are rare but highly aggressive tumors predominantly affecting children. The clinical course, imaging characteristics and histopathological features of meningeal sarcomas in two pediatric patients are presented. Furthermore, we critically discuss the new WHO classification of these entities comparing them to older descriptions.

In a 6-year-old girl, a cranial computed tomography (CT) scan was performed, after a mild head trauma, showing a parieto-occipital hemorrhage. One month later, a circumscribed mass adhering to the meninges and with central areas of hemorrhage was evident on magnetic resonance imaging (MRI) scans. Brain biopsy revealed a polymorphocellular sarcoma originating from the leptomeninges and infiltrating the brain.

In an 8-year-old girl, who presented with headaches and vomiting, several MRI-examinations were inconspicuous for nearly one year until the latest MR-scan revealed a diffuse contrast enhancement of the leptomeninges of the whole brain and spinal canal. After open biopsy, primary leptomeningeal sarcomatosis was diagnosed.

Although CT and MRI did not allow a specific diagnosis in both cases the exact visualization of the extent of the tumor and/or meningeal involvement was possible. Since there are no specific imaging criteria to differentiate meningeal sarcoma from other solid brain tumors or from other tumoral or inflammatory meningioses brain biopsy is indispensable. In order to avoid misinterpretations and delays of therapy, early open brain biopsy or surgical resection of the lesion is necessary in cases of unclear brain masses, especially of unclear meningeal processes. Due to the low number of cases published so far, the biological behavior and clinical management of this tumor entity still awaits further investigation.