Primary Intracranial Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor Mimicking Meningioma: A Case Report and Literature Review

Abstract

Background: Primary intracranial Ewing sarcoma (ES)/peripheral primitive neuroectodermal tumors (pPNETs) are extremely rare malignancies, which arise in children and adolescents, with only 9 cases reported in patients over 30 years of age. Due to its rarity, MRI features and treatment strategies for primary intracranial ES/pPNETs remain unclear. The purpose of this study was to explore the clinical features, imaging findings, pathological characteristics, different diagnoses, treatment, and prognosis of cerebellar liponeurocytoma in adults. Case Description: A 55-year-old female was admitted to the hospital with memory decline over 1 month, which aggravated in the last 2 weeks. MRI showed a 4.3 × 6.5 × 3.5 cm heterogeneous large mass in the left frontal lobe with mild peritumoral edema. The mass was successfully removed under neuronavigation and electrophysiological monitoring. The entire mass was removed, and postoperative pathology indicated an ES pPNET diagnosis, with an EWSR1 gene rearrangement. Subsequently, the patient underwent disciplinary radiotherapy. Conclusion: The diagnosis of primary intracranial ES/pPNETs depends on the comprehensive consideration of histological examination, immunohistochemical analysis, and genetic detection. Gross tumor resection combined with radiotherapy and chemotherapy might be the most beneficial treatment.

Primary intracranial peripheral primitive neuroectodermal tumor in an adult patient with aphasia: a rare case report

“…PNETs occur primarily in children and adolescents; the median age at initial diagnosis is about 15 years of age with a slight male predisposition [1,5]. The age of our case does not correspond to the common age of intracranial PNETs, which adds to the importance of our case.…”

“…Primary intracranial primitive neuroectodermal tumors (PNETs) are extremely rare malignancies that usually originate from the meninges and show varying degrees of neuroectodermal differentiation [1][2][3][4]. About 66 cases of these tumors have been reported so far.…”

“…They had a high propensity for local recurrence and distant metastasis. Intracranial PNETs commonly affect children and adolescents [1,2,5]. It is noteworthy that the incidence of PNETs in adults is exceedingly rare.…”

“…It is noteworthy that the incidence of PNETs in adults is exceedingly rare. Only about ten cases of these tumors have been reported over the age of 33 years [1,2]. Fusion of the EWSR1 gene with a member of the gene family is considered the primary cause of the PNETs [1,4].…”

“…Only about ten cases of these tumors have been reported over the age of 33 years [1,2]. Fusion of the EWSR1 gene with a member of the gene family is considered the primary cause of the PNETs [1,4]. Due to the scarcity of cases of patients with PNETs, the clinical and imaging features, treatment and prognosis are not clear [1,2].…”