Primary meningeal sarcomas in two children

Primary meningeal sarcomas are rare but highly aggressive tumors predominan tly affecting children. The clinical course, imaging characteristics and hi stopathological features of meningeal sarcomas in two pediatric patients ar e 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 mon th later, a circumscribed mass adhering to the meninges and with central ar eas of hemorrhage was evident on magnetic resonance imaging (MRI) scans. Br ain biopsy revealed a polymorphocellular sarcoma originating from the lepto meninges and infiltrating the brain. In an 8-year-old girl, who presented with headaches and vomiting, several M RI-examinations were inconspicuous for nearly one year until the latest MR- scan revealed a diffuse contrast enhancement of the leptomeninges of the wh ole brain and spinal canal. After open biopsy, primary leptomeningeal sarco matosis was diagnosed. Although CT and MRI did not allow a specific diagnosis in both cases the ex act visualization of the extent of the tumor and/or meningeal involvement w as possible. Since there are no specific imaging criteria to differentiate meningeal sarcoma from other solid brain tumors or from other tumoral or in flammatory meningioses brain biopsy is indispensable. In order to avoid mis interpretations and delays of therapy, early open brain biopsy or surgical resection of the lesion is necessary in cases of unclear brain masses, espe cially of unclear meningeal processes. Due to the low number of cases publi shed so far, the biological behavior and clinical management of this tumor entity still awaits further investigation.