Hypothalamic Extraventricular Neurocytoma (EVN) in a Pediatric Patient: A Case of EVN Treated with Subtotal Removal Followed by Adjuvant Radiotherapy

Abstract

Extra ventricular neurocytoma (EVN) is a rare brain tumor with histologic features similar with a central neurocytoma, but located outside of the ventricular system. In this study, we present an unusual case of hypothalamic EVN in a 14-year-old patient. The patient underwent subtotal removal and had tumor relapse. The patient was then treated using intensity modulated radiation therapy, and the tumor remained stable for 24 months. This case report may be important in that this is the first pediatric case of EVN located in the hypothalamic region. EVN has similar radiologic features with pilocytic astrocytomas and therefore a hypothalamic EVN may be misdiagnosed as a hypothalamic glioma. Also, the pathologic-radiologic-clinical correlation of EVN located in the hypothalamic area may be different from that of EVNs originating from other usual sites.

Cerebellar Neurocytoma with Excellent Response to Radiotherapy

“…Neurocytomas with high FDG uptake also showed an increased proliferative index associated with atypical histological features (11). In the current case, the tumour showed benign features with Ki67 < 1% (12). However, the tumour grew rapidly after needle biopsy.…”

Pediatric diencephalic tumors: a constellation of entities and management modalities

“…On MRI, the lesion was T1 isointense, T2 hyperintense, with marked peripheral enhancement and central necrosis. The patient was treated with subtotal resection, and histopathology confirmed a WHO grade IV neoplasm with anaplastic hyperchromatic cells, atypical mitoses, and vascular proliferation (28).…”

Whole Exome Sequencing Identifies PHF14 Mutations in Neurocytoma and Predicts Responsivity to the PDGFR Inhibitor Sunitinib

“…Neurocytomas are rare, represent 0.1–0.5% of primary intracranial tumors, and typically affect young adults [ 1 ]. Neurocytomas are categorized based on their location, namely central neurocytomas (CN) which involve the septum pellucidum, fornix, or lateral ventricular walls; whereas extraventricular neurocytomas (EVN) occur in the frontal or temporal lobes, spinal cord brain stem, and rarely the sellar region [ 2 , 3 , 4 ]. Defined as Grade II by the latest world health organization (WHO) classification, neurocytomas usually exhibit a benign course with good outcome if gross-total surgical resection (GTR) is achieved.…”