Pediatric spinal pilomyxoid astrocytoma (PMA) is an extremely rare tumor that merits recognition as a specific, unique entity. The authors present the case of an intramedullary PMA in the thoracic spinal cord of an 11-year-old boy who presented with back pain, scoliosis, and multiple lung nodules. The patient underwent T5–11 laminoplasty and near-total resection of the spinal tumor. The final pathological diagnosis was WHO Grade II PMA. The patient did well for 14 months until the tumor progressed both clinically and radiographically. A literature review focusing on the clinical characteristics, histology, and treatment of PMAs provides a better understanding of these rare lesions. Because of the small number of cases optimal treatment guidelines have not been established, but gross-total resection and adjuvant chemotherapy with alkylating agents appear to confer a better long-term prognosis. Pediatric patients with PMAs can remain recurrence free at least 5 years after surgery, although these tumors may disseminate or dedifferentiate into more malignant gliomas. Recognition of intramedullary PMA as a unique entity in children is vital to the development of specific surgical and adjuvant treatment regimens.
Abbreviations used in this paper:GTR = gross-total resection; PMA = pilomyxoid astrocytoma; STR = subtotal resection.
Address correspondence to: Jay K. Riva-Cambrin, M.D., Department of Neurosurgery, Division of Pediatric Neurosurgery, University of Utah, 100 Mario Capecchi Dr., Salt Lake City, UT 84113. email: firstname.lastname@example.org.
Please include this information when citing this paper: published online September 20, 2013; DOI: 10.3171/2013.8.PEDS1397.
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