Case report: Primary intracranial EWs/PNET in adults: Clinical experience and literature review

Abstract

IntroductionAdult primary intracranial Ewing sarcomas (EWs)/primitive neuroectodermal tumors (PNETs) are extremely rare, with only 30 patients published before us. The imaging features and treatment strategies of primary intracranial EWs/PNETs are unclear due to its rarity. The aim of this study was to investigate the clinical features, imaging findings, treatment, survival analysis, and prognosis of adult EWs/PNETs, and a systematic review was conducted based on the patient we treated and published literature.Case descriptionA 19-year-old male patient suffered from head pain due to an accidental fall on a motorcycle that occurred more than 10 days before going to the hospital, and underwent computed tomography (CT) examination; it was found that the left temporo-occipital fossa was occupied. Magnetic resonance imaging (MRI) was recommended to understand the nature of the lesion, and the result showed that it has a high probability of being a meningioma. He underwent surgical removal of the mass under general anesthesia, and surprisingly, postoperative pathology revealed EWs/PNET. The disease has a high degree of malignancy, and the patient developed multiple metastases throughout the body 5 years after surgery.ConclusionPrimary intracranial EWs/PNETs in adult patients are rare, of which imaging findings should be considered as one of the differential diagnoses of meningioma, hemangiopericytoma, and malignant triton tumor. Larger solid-cystic masses with septum-like enhancement may be relatively specific imaging findings of intracranial EWs/PNETs. The prognosis of primary adult intracranial EWs/PNETs is poor. Radical tumor resection combined with radiotherapy and chemotherapy is currently the main and possibly the most effective treatment method.

A case of giant Ewing's sarcoma (EES)/primitive neuroectodermal tumor (PNET) of the cervicothoracic junction in children with incomplete paralysis of both lower limbs: Case report and literature review

“…Ewing sarcoma (ES) and primitive neuroectodermal tumors (PNET) are collectively referred to as ES/PNET because they share the same genetic and histological features (1). ES/PNET are highly malignant small round cell tumors with multidirectional differentiation potential and can be classified as intraosseous and extraosseous depending on their origin in bone or soft tissue (2).…”

Fluoro-18-fluorodeoxyglucose positron emission tomography/computed tomography detects Ewing’s sarcoma of the larynx with multiple distant bone metastases: a case report and literature review

“…According to the World Health Organization's 5th classification of bone and soft-tissue tumors in 2020, EWS of bone, primitive neuroectodermal tumors, extraskeletal EWS, and Askin's tumors are referred to collectively as the EWS family; extraskeletal EWS are rare, accounting for only 15%-20% of the EWS family (2). The most common extraskeletal EWS affect the paravertebral soft tissue, lung, stomach, kidney, and bladder; the larynx is a rare site of origin (2)(3)(4)(5). The clinical features of laryngeal EWS are associated with tumor progression, but no clinical manifestation is obvious when the tumor is small.…”