Abstract
Neuroblastomas are the most common extracranial solid tumor in the pediatric age group (~8%-10% of childhood neoplasms). Most cases of intracranial neuroblastomas occur due to metastasis from some primary extracranial sites and are known as secondary neuroblastomas. However, the occurrence of primary central nervous system neuroblastomas (PCN-NB) is very rare, and only a few cases and case series have been reported in the literature. PCN-NB is mainly an intra-axial pathology, and extra-axial involvement is mainly due to metastasis from some extracranial primary site with involvement of the skull bone. Herein we report a case of a 23-year-old female having a large extra-axial space-occupying lesion in the right frontal region that was mimicking a meningioma, and surprisingly the histopathology was suggestive of a supratentorial neuroblastoma. A right frontal craniotomy was made, and Simpson's grade 1 excision of the tumor was done. The excised tissue was sent for histopathological examination. PCN-NB located extra-axially are extremely rare to occur. Due to inconsistent radiological imaging, it becomes very difficult to diagnose these tumors preoperatively, and these should be kept in mind as one of the differential diagnoses of extra-axial intracranial space-occupying lesions. Histopathological examination is crucial in diagnosing the intracranial neuroblastomas.