Contributed by Janet Yoon MD1; Andrew Martin MD2; Denise Malicki MD, PhD3; Robert Newbury MD3; John Crawford MD, MS4
Departments of 1Pediatrics, Division of Hematology/Oncology, 3Pathology, 4Neurosciences
Rady Children's Hospital San Diego and University of California San Diego, San Diego, CA, USA.
Department of 2Hematology/Oncology, University of Texas Southwestern, Dallas, TX, USA
A 16-month-old boy presented to his pediatrician following a 3 week history of left head tilt and a 3 day history of cessation of crawling. Developmental history was significant for delays in sitting independently (8 months) and crawling (12 months), necessitating physical therapy for delayed gross motor milestones. Birth history and all other past medical history were unremarkable. Neurologic examination was positive for a mild left head tilt and lower extremity proximal muscle weakness on ventral suspension. Muscle bulk, tone, sensation, reflexes, and coordination were all within normal limits. MRI of the spine showed an expansile, enhancing, intramedullary mass from C2-T3 with an associated syrinx above and below the lesion (Fig 1). He subsequently underwent complete C3 to T3 laminectomies for spinal cord decompression and tumor resection.
Microscopic examination revealed small fragments of neuroglial tissue with increased cellularity in a heterogeneous distribution. Medium power- 200x (Fig 2) and high power- 600x (Fig 3) views demonstrated moderately cellular proliferation of monomorphic, process-forming cells in a basophilic proteinaceous background with angiocentric nuclei. Mitoses were not identified. What is your diagnosis?