Presentation
Patient with adequate psychomotor development, who presents headache in the bilateral pterional region and bilateral amaurosis, who has undergone surgery on two occasions with partial resection of the tumor and placement of a peritoneal ventricular shunt valve due to presenting hydrocephalus (there are no pre-surgical imaging studies).
Patient Data
In the suprasellar region in the visual pathway, involving optic nerves, chiasm, and optic tracts up to the region of the right external geniculate ganglion, an extra-axial lesion of irregular shape, lobulated edges, heterogeneous at the expense of some areas of cystic change is observed (it does not suppress in FLAIR ), with a predominance of hypointense signal in the white matter on T1, hyperintense on T2 and FLAIR, with some areas of magnetic susceptibility artifact in relation to hemosiderin.
Homogeneous enhancement in the solid areas. It is located in a superior sagittal line to the sella turcica, obliterating the suprasellar cistern extending towards the third ventricle, lateral ventricles, caudally occupying the prepontine and interpeduncular cistern, with invasion of the anterior portion of the right lenticular nucleus and mass effect of both nuclei and peduncles.
Surgical changes characterized by frontal craniectomy with malacia surrounded by gliosis that runs deeply until reaching the anterior horn of the right lateral ventricle, with which it generates communication with the subarachnoid space, compatible with porencephaly, are identified. Occipital trephines with linear trajectory shunt valves with flared distal tips, one in each posterior ventricular horn.
Case Discussion
Pilocytic astrocytomas are one of the main causes of death in children as they are the most common central nervous system tumor in these age groups.
Approximately half of all cases of pilocytic astrocytomas occur in the cerebellum and the other half in the entire central nervous system Those in the optic pathway are most frequently seen in the setting of NF1.
This patient had a biospy/resection and the diagnosis of pilocytic astrocytoma was confirmed histologically.
The 2021 World Health Organization classification of central nervous system tumors reflects the discovery of genetic alterations that underlie many central nervous system neoplasms, including pilocytic astrocytoma. Unfortunately, resources are not readily available in all countries, and therefore it is not possible to obtain certainty molecular diagnoses in all cases.
Unable to process the form. Check for errors and try again.