Sudden death due to primary diffuse leptomeningeal gliomatosis

Tumors of the central nervous system are an unusual cause of sudden death. This report describes the sudden death of a presumed healthy 28-year-old woman from primary diffuse leptomeningeal gliomatosis. She presented to an emergency room with headache and vomiting, subsequently became unresponsive and was pronounced dead 14 h later. Autopsy revealed a diffuse extensive infiltrate of well-differentiated astrocytoma in the leptomeninges of the brain and spinal cord without an underlying parenchymal tumor. Primary diffuse leptomeningeal gliomatosis is a rare tumor that arises within the leptomeninges from small neuroglial heterotopic rests that undergo neoplastic transformation. Grossly, this tumor can mimic leptomeningeal carcinomatosis, pachymeningitis, tuberculosis, sarcoidosis, and fungal infections. However, the histologic features of primary diffuse leptomeningeal gliomatosis should allow it to be readily distinguished from grossly similar conditions. The mechanism of death in this case is most likely tumor obstruction of cerebrospinal fluid outflow resulting in the usual complications seen with increased intracranial pressure. Although this tumor is aggressive and is associated with a rapidly progressive fatal course, it has not been previously associated with sudden death.