Pediatric spinal pilomyxoid astrocytoma.
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.
Department of Neurosurgery, Clinical Neurosciences Center, University of Utah, Salt Lake City, Utah.
Sarah T
ST
Garber
Robert J
RJ
Bollo
Jay K
JK
Riva-Cambrin
D018906:Antineoplastic Agents, Alkylating / Q000008:administration & dosage; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001254:Astrocytoma / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000556:secondary / Q000601:surgery; D001416:Back Pain / Q000209:etiology; D017024:Chemotherapy, Adjuvant; D002648:Child; D006801:Humans; D008175:Lung Neoplasms / Q000556:secondary; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000628:therapy; D011379:Prognosis; D012600:Scoliosis / Q000209:etiology; D013120:Spinal Cord Neoplasms / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology / Q000601:surgery
D018906:Antineoplastic Agents, Alkylating