Abstract

Background: Cerebellar pilocytic astrocytoma (PCA) is one of the few CNS tumors that can be cured with gross-total removal (GTR). In this series, we had 39 patients diagnosed with cerebellar PCA, 27 patients (70%) had GTR, and mean follow-up period was 62 months with no tumor recurrence.Objective: To assess the long-term outcome of childhood cerebellar PCA treated at our institute during the period 2000-2020 and to highlight our surgical protocol.Methodology: Retrospective review of all patients under 18 years of age who were diagnosed with cerebellar PCA and had surgical excision between 2000 and 2020 at the Medical City of King Saud University.Results: The study included 39 patients: 17 males and 22 females, the mean age was 8.4 years. Radiologically, the tumor was solid in eight patients, cystic in 15 patients, and mixed components were found in 16 patients. The lesion was located in the right cerebellar hemisphere in 12 patients, left cerebellar hemisphere in five patients, and midline 22 patients. The tumor size ranged from 2 to 7 cm in its greatest diameter, it was <5 cm in 13 patients and >5 cm in 26 patients. Thirty-one patients had preoperative hydrocephalus. GTR of the tumor was achieved in 27 patients and subtotal resection (STR) was done in 12 patients, 18 patients required permanent ventriculoperitoneal (V-P) shunt, and five patients had postoperative radiotherapy. Postoperative complications included infection in two patients, cerebellar mutism in two patients, and significant neurologic disability in four patients. The duration of follow-up ranged from 0 to 240 months (mean follow-up period: 62.0 months). The outcome at 10 years was good in 30 patients, fair in four patients, poor in four patients, and one patient died. Recurrence was documented in nine patients, seven of them had GTR and two had STR.Conclusion: GTR, if achievable, is curative for childhood cerebellar PCA. Many posterior fossa surgical complications could be avoided with watertight dural closure. Although new dural substitutes are available we prefer using autologous grafts (pericranium). It is easy to harvest pericranial graft from the external ventricular drain (EVD) site. The insertion of EVD synchronously with GTR of the tumor and gradual weaning of EVD could avoid the insertion of V-P shunt.

Highlights

  • Pilocytic astrocytoma (PCA) was first described by Cushing in 1931 as an independent pathologic entity [1]

  • Recurrence was documented in nine patients, seven of them had gross-total removal (GTR) and two had subtotal resection (STR)

  • About 80% of pilocytic astrocytoma (PCA) are found in the posterior fossa, it can arise in the cerebellum, brain stem, optic tract, thalamus, and hypothalamus

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Summary

Introduction

Pilocytic astrocytoma (PCA) was first described by Cushing in 1931 as an independent pathologic entity [1]. The presenting symptoms are attributed to increased intracranial pressure (ICP) including headache, nausea, vomiting, papilledema, and cerebellar signs. It is either a solid tumor, cystic with an enhancing mural nodule, or a mixture of both [7,8,9,10]. Cerebellar pilocytic astrocytoma (PCA) is one of the few CNS tumors that can be cured with gross-total removal (GTR). In this series, we had 39 patients diagnosed with cerebellar PCA, 27 patients (70%) had GTR, and mean follow-up period was 62 months with no tumor recurrence

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