Pediatric spinal pilomyxoid astrocytoma

Case report

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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.

Article Information

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: neuropub@hsc.utah.edu.

Please include this information when citing this paper: published online September 20, 2013; DOI: 10.3171/2013.8.PEDS1397.

© AANS, except where prohibited by US copyright law.

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Figures

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    Sagittal T1-weighted (A) and T2-weighted (B) MRI studies obtained without contrast, and a T1-weighted MRI study (C) obtained after addition of contrast material showing enhancing midthoracic spinal cord tumor with associated cord syrinx.

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    Axial (A and B) and coronal (C) T1-weighted MRI studies obtained after addition of contrast material showing enhancing nodules (arrows) in both lungs and thoracic dextroscoliosis secondary to the intramedullary tumor.

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    Sagittal T1-weighted (A) and T2-weighted (B) MRI studies obtained without contrast, and a T1-weighted (C) MRI study obtained after addition of contrast material showing no residual areas of nodular enhancement, decreased size of cord syrinx, but with mild enhancement of the cavity wall (C).

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    A: Medium-power magnification photomicrograph showing a hypercellular tumor with areas of increased vascularity. B: Highpower magnification view showing mucinous background and piloid cells with few mitotic figures and an absence of Rosenthal fibers. C: High-power magnification view showing mucinous background and piloid cells with a few mitotic figures and an absence of Rosenthal fibers. H & E, original magnification ×40 (A) and ×100 (B and C).

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    Sagittal (left) and axial (right) T1-weighted MRI studies obtained after addition of contrast material showing recurrence of disease (arrow) in the resection bed at approximately the T5–6 level.

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