A rare case of atypical spinal neurocytoma with EGFR mutation in a 12-year-old boy
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CASE REPORT
A rare case of atypical spinal neurocytoma with EGFR mutation in a 12-year-old boy Beibei Yu 1,2
&
Jiatong Li 2 & Linkai Jing 1,2 & Weitao Man 1,2 & Guihuai Wang 1,2
Received: 13 August 2020 / Accepted: 30 September 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Spinal neurocytoma (SN), although frequently reportedly as tumors of the central nervous system (CNS), are a distinct class of tumors, which can achieve a better prognosis following subtotal or gross total tumor resection. Nonetheless, even with the premise of successful treatment after tumor resection, poor prognosis after treatment due to the SN high proliferation index (typically known as atypical SN) have been reported. Over the past two decades, atypical SN was only reported in four pediatric cases, amidst the lingering controversy surrounding its postoperative adjuvant therapy. Thus, herein, we report a unique case of atypical SN with epidermal growth factor receptor (EGFR) amplification mutation in a 12-year-old boy. We, however, also highlighted the significance of radiotherapy and target therapy for patients with SN. Keywords Spinal neurocytoma . Atypical . EGFR . Target therapy
Background Extraventricular neurocytoma (EVN, WHO grade II) is a sporadic neuronal neoplasm, accounting for 0.2–0.5% of primary tumors in the CNS [1]. Given that the EVNs are predominantly located in the frontal lobe, only 25 spinal cases were reported in the literature [2]. Although necessary clinical information is scarce in the literature, SN is often known to express better biological behaviors, which is mostly achieved with gross total resection (GTR) in most cases [3]. Notably, a subset of SNs with a higher Ki-67 index presents a higher probability of recurrent and relatively adverse outcomes [4]. Thus, these SNs subsets, and the relative adverse outcomes Jiatong Li is the Co-first author * Guihuai Wang [email protected] Beibei Yu [email protected] Jiatong Li [email protected] 1
Department of Neurosurgery, Changgung Hospital, Clinical Institute for Neuroscience, Medical Center, Tsinghua University, Beijing 102218, China
2
School of Clinical Medicine, Tsinghua University, Beijing 100084, China
prompted the introduction of adjuvant radiotherapy and chemotherapy to improve the prognosis of atypical SN. However, due to the heterogeneous responses to various treatments, the standard therapeutic strategy for SN is yet to be defined. Here, we report an atypical intramedullary neurocytoma with epidermal growth factor receptor (EGFR) mutation, not detected in previous studies, thus, providing evidence-based potentials for targeted therapy in the management of a child with SN.
Case report A 12-year-old boy was admitted to our institution (Beijing Tsinghua Changgung Hospital) with a 1-year history of right-hand weakness with a 5-day acute progression of right upper arm weakness. Upon physical examination, his muscle strength was graded 4/5 in the right upper extremity precisely at the C5/C6/C7 region, while a grade 3/5 wa
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