Low-grade astrocytoma with neuraxis dissemination at diagnosis

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✓ Little is known about low-grade astrocytoma with neuraxis dissemination at diagnosis. A review of medical records identified this phenomenon in eight of 150 pediatric patients evaluated between 1985 and 1994 for histologically confirmed low-grade astrocytoma. These patients (five male and three female) ranged in age from 5 months to 20 years (median 8 years). Symptoms of neuraxis disease were minimal or absent. Primary tumor sites were the hypothalamus in four cases, brainstem/spinal cord in three, and temporal lobe in one. Patterns of dissemination (evaluated by computerized tomography and/or magnetic resonance imaging techniques) appeared to be related to the primary site: hypothalamic tumors metastasized along the ventricular cerebrospinal fluid pathways, and tumors in other locations disseminated along subarachnoid pathways. Following initial treatment with chemotherapy (in three), partial resection (in one), radiation therapy (in three), and chemotherapy plus irradiation (in one), four patients required salvage therapy for progressive or recurrent disease. Seven of the eight patients are alive with stable or progressive disease 6 to 105 months postdiagnosis (median 15 months). Low-grade astrocytoma with initial neuraxis dissemination is responsive to chemotherapy and radiation, a proportion showing periods of stable disease. The optimum therapy or combination of therapies remains unclear.

Article Information

Address reprint requests to: Amar Gajjar, M.D., St. Jude Children's Research Hospital, 332 North Lauderdale, Memphis, Tennessee 38101–0318.

© AANS, except where prohibited by US copyright law.

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    Case 3. Contrast-enhanced T1-weighted magnetic resonance image showing an enhancing hypothalamic tumor impinging into the third ventricle. Enhancing metastases are seen coating the ventral aspect of the brainstem and spinal cord, the quadrigeminal plate cistern, and the cerebellum.

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    Case 3. T1-weighted magnetic resonance images of the thoracolumbar spine before (left) and after (right) contrast enhancement, showing multiple nodular enhancing metastases coating the spinal cord.

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    Case 3. Subtraction image of a contrast-enhanced T1-weighted magnetic resonance image showing cerebrospinal fluid enhancement ventral to the pons and medulla.

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    Case 5. Contrast-enhanced computerized tomography showing nodular leptomeningeal metastases from a spinal cord tumor in the interpeduncular fossa and the ambient cistern. There are also nodular metastases in the sylvian fissures bilaterally.

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