Contributed by Shawn L. Hervey-Jumper, MD1; Anthony C. Wang, MD1; Andrea N. Yates, BS1
Patricia L. Robertson, MD2; Karin M. Muraszko, MD1; Hugh J.L. Garton, MD, MHSc1; Mila Blaivas, MD, PhD3
Departments of 1Neurosurgery, 2Pediatrics, and 3Pathology, University of Michigan, Ann Arbor, Michigan, USA
CLINICAL HISTORY AND IMAGING STUDIES
An 8-year-old boy with no significant past medical history presented to the emergency department following 2 weeks of progressive headaches, fatigue, and vomiting. On physical examination, the patient had no fever, or focal motor or sensory neurological deficits. Initially diagnosed with an H1N1 viral upper respiratory and gastrointestinal infection, he was discharged home with conservative management. After failure to improve over the next several days, a superimposed bacterial infection was suspected. He was given a course of oral antibiotics, which failed to improve his symptoms. Imaging revealed a large, heterogenously enhancing mass measuring 5.6 x 4.7 x 5.2 cm, which contained partially hemorrhagic material centered within the left thalamus extending into the body of the lateral ventricle (Figures 1, 2, 3, 4). Gross total resection of the tumor was confirmed by postoperative imaging. Staging revealed no metastases. The patient was discharged home 1 week later.
Grossly, the tumor submitted for neuropathological evaluation consisted of multiple pieces of friable, tan tissue fragments altogether measuring 2 x 2 x 0.5 cm.
Microscopically, the tumor was formed by pleomorphic, frequently multinucleated bizarre cells with numerous mitotic figures and multiple abnormal spindles (Figure 5), multifocal tumor necrosis, calcifications, and exuberant microvascular proliferation (Figure 6). Immunostaining of the sections showed many areas of neoplasm staining positively for synaptophysin (Figure 7), and occasional cells positive for glial fibrillary acidic protein (GFAP) (Figure 8) and vimentin. Positive Tuj1 staining was seen in some of these areas as well. Few cells were positive for S-100 protein, and none for NeuN or neurofilament protein. P53 as well as epidermal growth factor receptor (EGFR) (Figure 9) showed robust multifocal staining. MIB-1 proliferation index was high, approaching close to 100% in some areas.