Surgery of spinal cord pilocytic astrocytoma complicated by leptomeningeal dissemination to the brain and spine and rapid progression

Abstract

Pilocytic astrocytoma (PCA) is a common benign astrocytic tumor in children that usually arises in the cerebellum, brainstem, hypothalamic region, or optic pathways. It can also arise in the spinal cord as an intradural intramedullary PCA. In rare cases, it presents with leptomeningeal dissemination, which leads to a poor clinical outcome. We present a rare case of spinal cord pilocytic astrocytoma with leptomeningeal dissemination to the brain and spine with rapid progression. We present a 13-year-old girl with progressive weakness of her right arm. An intramedullary hypervascular neurogenic tumor at the C3–C6 level was identified by magnetic resonance imaging (MRI) and surgical excision was performed. PCA was proven by pathology. The tumor recurred 3 months later and an intramedullary polycystic astrocytoma at the C1–T1 level was confirmed by MRI. Total tumor resection was performed and pathological characteristics again confirmed PCA. However, diplopia with blurred vision was noted 1 month after the last surgery, which was accompanied by signs of increased intracranial pressure (IICP). MRI indicated leptomeningeal enhancement in the brain and tumor seeding along the spinal cord. Chemotherapy and radiotherapy were administered, but the clinical course rapidly deteriorated and the patient died of respiratory distress. Although the prognosis for low-grade astrocytomas is good in most reported studies, this case showed leptomeningeal dissemination with a rapid course that resulted in death. A fatal outcome might be anticipated when encountering difficult intramedullary spinal cord lesions, as in our case.