Pediatric Cerebellar Glioblastoma Multiforme presenting as Supra and Infratentorial Acute Disseminated Encephalomyelitis/ Gliomatosis Cerebri: A Case Report
Patrick B Senatus M.D., Ph.D.1, Shearwood McClelland III B.A.1, Kurenai Tanji M.D.2, Judy Huang M.D.3, Neil Feldstein M.D.1
Departments of 1Neurosurgery and 2Neuropathology, Columbia University, College of Physicians and Surgeons, New York, NY
3Department of Neurosurgery, Johns Hopkins School of Medicine, Baltimore, MD
Gliomatosis cerebri represents a malignant transformation of glial cells with preservation of existing cellular architecture. Radiographically the distinction between gliomatosis cerebri and demyelinating processes is not entirely clear. We report the first presentation of pediatric cerebellar glioblastoma multiforme (GBM) presenting simultaneously as supra and infratentorial glial transformation initially resembling acute disseminated encephalomyelitis (ADEM).
A previously healthy 5-year-old Caucasian boy presented with 4 days of rapidly progressive neurological decline characterized by ataxia, left facial weakness, and nystagmus. Recent history was remarkable for recurrent upper respiratory infections without sequelae during the past month, and a sick contact with a headache-like illness two weeks prior to admission. Based on his symptomatolgy, recent history and radiographic findings (FIGURE 1), he was assigned a presumptive diagnosis of ADEM, and was treated with antibiotics, antivirals, steroids, and intravenous immune globulin (IVIG) with modest improvement of his symptoms (manifesting as improvement of nystagmus, but persistence of left facial palsy).
Despite therapy, the patient underwent progressive neurological decline over the next 2 months. Repeat imaging (FIGURE 2) demonstrated a discrete cerebellar lesion with effacement of the 4th ventricle, necessitating ventriculostomy for worsening obstructive hydrocephalus and suboccipital craniectomy for tissue diagnosis.
Pathology revealed a highly cellular infiltrative glial neoplasm with features of nuclear atypia, numerous mitotic figures, pseudopalisading tumor necrosis, and endothelial proliferation. From these findings, the diagnosis of GBM of the cerebellum was made.
In summary, this case represents the first report of a supra and infratentorial gliomatosis presenting as ADEM. This case underscores the need to recognize gliomatosis cerebri as part of the differential diagnosis for ADEM. If gliomatosis cerebri is diagnosed early, there are existing treatments, such as whole brain irradiation, that have been shown to promote preservation of lifespan.