Spinal cord pilocytic astrocytoma with leptomeningeal dissemination to the brain

Abstract

Leptomeningeal dissemination of low-grade spinal cord gliomas is an uncommon event. The authors report a unique case of leptomeningeal dissemination of a spinal cord pilocytic astrocytoma (PCA) to the intracranial cerebral subarachnoid spaces in a child. A 2-year-old boy presented with a loss of balance and the inability to walk or stand. An intradural intramedullary spinal cord tumor was identified, and the lesion was subtotally resected and diagnosed by the pathology department to be a PCA. Subsequently, the patient had recurrences of the intradural intramedullary tumor at 6 months and 2 years after his original presentation. He underwent a repeated resection of the recurrent tumor and fenestration of an associated syrinx on both occasions. The pathological characteristics of the reresected tumor remained consistent with those of a PCA. Postoperative imaging after his last surgery revealed diffuse intracranial leptomeningeal dissemination into the cisternal space surrounding the midbrain, the suprasellar region, and the internal auditory canal, as well as nodular subarachnoid disease in the upper cervical region. The patient then underwent chemotherapy, and total spine magnetic resonance (MR) imaging 2 months later demonstrated stability in the size of the spinal cord tumor and a decrease in the associated syrinx. However, an MR image of the head demonstrated two new areas of supratentorial subarachnoid leptomeningeal spread of the primary spinal cord tumor at the 2-month follow-up examination. At the 6-month follow-up examination, MR imaging of the head and spine demonstrated stable metastatic disease. This case illustrates a unique instance of supratentorial leptomeningeal dissemination of an intramedullary spinal cord PCA in a child.

A retrospective observational study on the treatment outcomes of 26 patients with spinal cord astrocytoma including two cases of malignant transformation

“…), and Ki-67 was 30 % in tumor cells. Similar to a previous study in which malignant spinal cord astrocytoma usually disseminated following neuraxis [9] and a recent study on low-grade astrocytoma leptomeningeal dissemination to the brain [30], our case also had EGFR positivity and neuraxis dissemination with malignant transformation. Promoter methylation of the MGMT gene and IDH1 mutation were not observed.…”

Heterogeneity of histopathological presentation of pilocytic astrocytoma – diagnostic pitfalls. A review

“…Clinical behaviour is often unpredictable, and PAs may recur locally after incomplete resection or disseminate to the leptomeninges, especially when they are located in the hypothalamic region [1]. It must be noted that recurrences of PA are not associated with histological malignant progression.…”

“…Leptomeningeal dissemination of the cerebellar tumours into the spinal cord might occur many years after primary surgery [45,66,78]; less commonly the meningeal seeding appears at the initial presentation [67]. Spread into the spinal meninges can be observed in PAs of the spinal cord [1,9] and optic chiasm [4,48]. Extremely rarely, spinal cord PA can spread via the cerebrospinal fluid pathway (CSF) to the cerebral meninges [69].…”

Intracranial Metastases of Cervical Intramedullary Low-Grade Astrocytoma without Malignant Transformation in Adult

“…Cerebral metastases of a primary spinal astrocytoma have rarely been reported 1,6,13,17,19,22,24,25) . Of the spinal cord gliomas reported to have metastasized to the brain, most were anaplastic astrocytomas or glioblastoma multiforme 7,13,19,20,24,25) .…”

“…Of the spinal cord gliomas reported to have metastasized to the brain, most were anaplastic astrocytomas or glioblastoma multiforme 7,13,19,20,24,25) . Seeding to cranial sites from intramedullary low-grade astrocytoma has been reported in only a few cases; these have reported intracranial spread of intramedullary pilocytic astrocytoma without malignant transformation in a child 1,17) and with malignant transformation in an adult 6,19,24) . We report the case of a metastatic spread to the cerebellum of a low-grade astrocytoma without malignant transformation in a 52 year-old male.…”