Primary Ewing’s sarcoma affecting the central nervous system: a review and proposed prognostic considerations

Primary Ewing sarcoma of the cavernous sinus

“…Soft-tissue ES in the CNS is considered part of the ES/ pPNET family because of their identical genetic features; however, these tumors should not be considered the same as CNS PNETs as the latter tumors are not only different genetically but also require different treatment paradigms and have a different prognosis from ES/pPNETs [7,10]. The lesion in our case showed marked expression of the surface glycoprotein CD99.…”

“…Although these tumors typically form in bone, they also can originate in soft tissue [7]. Extraosseous ES can occur at several sites, including the chest wall, head, neck, soft tissues, and in any viscera [11,13].…”

“…Dramatic reduction with near resolution of tumor at 9 months CN cranial nerve, CO craniotomy, CS cavernous sinus, Chemo chemotherapy, GK gamma-knife, ICA internal carotid artery, IHC immunohistochemistry, L left, R right, RT radiotherapy, SOF superior orbital fissure, TS trans-sphenoidal ES, particularly extraosseous ES arising within the CNS [7]. Indeed, primary ES most often affects CNS structures when it arises in the calvarium or spinal column and causes a mass effect on adjacent neural or vascular structures [7].…”

“…Indeed, primary ES most often affects CNS structures when it arises in the calvarium or spinal column and causes a mass effect on adjacent neural or vascular structures [7]. Nevertheless, primary intracranial ES rarely has been reported throughout the CNS, including at the jugular foramen, in the cerebellopontine angle, and involving the frontal dura and tentorium [8].…”

Unusual clear cell, lymphoplasmacyte-rich, dural-based tumor with divergent differentiation: a tricky case mimicking a meningioma

“…Primary CNS neuroectodermal tumors are subdivided into central forms, including medulloblastomas, supratentorial primitive neuroectodermal tumors (cPNETs), ependymoblastomas, atypical teratoid/rhabdoid tumors and medulloepitheliomas, and peripheral primitive neuroectodermal tumors (pPNETs) [3,4]. Recently, Korshunov et al [5] published that embryonal tumors with abundant neuropil and true rosettes, ependymoblastomas, and medulloepitheliomas share molecular similarity and comprise a single clinicopathological entity, so it is likely that the above subtypes will not be retained in the upcoming WHO revision.…”