Ewing's Family of Tumors Involving Structures Related to the Central Nervous System: A Review

Abstract

This review consolidates information gleaned from several case reports and larger series on Ewing's sarcoma family of tumors (EFT) involving structures related to and found in the central nervous system (CNS). These tumors involve the skull, the spinal column, adjacent soft tissues, the meninges, and the brain. We have separated the cases by skull region and spinal column level, and we discuss the attendant differences in prognosis following treatment by neurosurgery, radiation, and chemotherapy. Light and electron microscopic features can be used to differentiate EFT from other small round blue cell tumors that involve the CNS (central primitive neuroectodermal tumor, lymphoma, etc.). Recent molecular and genetic findings in EFT provide new diagnostic methods. We conclude that EFT involving the CNS and adjacent structures is not so rare as previously stated and that the prognosis is more favorable, as a rule, than for the more common examples arising in the long bones and pelvis.

Sarcoma de Ewing extraósseo

“…Nessa ocasião, apresentava tumoração na região glútea e lombar esquerda e cicatriz de mais ou menos 2,5 cm correspondente ao local da drenagem realizada. Hadfield et al 13 descreveram diversos casos de sarcoma da família Ewing envolvendo estruturas do sistema nervoso, destacando-se o crâ-nio, a medula espinhal, tecidos adjacentes, as meninges e o cérebro.…”

A Peripheral Primitive Neuroectodermal Tumor with Generalized Bone Metastases in a Puppy

“…3,4,11,13 As a group, pPNETs have been clearly separated from classical PNETs of the peripheral nervous system (PNS) (e.g., neuroblastoma, ganglioneuroblastoma, and ganglioneuroma) by differences in their clinical, pathologic, and molecular genetic profiles. [1][2][3][4]8,11,13,15 Currently the EFT group includes the intraosseous pPNET (or Ewing's sarcoma), the extraosseous pPNET and the thoracopulmonary Askin's tumor. 3,4,13,15 The primary site of Ewing's sarcoma arises in the long tubular bones and pelvis with subsequent hematogenous metastases most commonly to lung, other bones, and the bone marrow.…”

“…3,4,13,15 The primary site of Ewing's sarcoma arises in the long tubular bones and pelvis with subsequent hematogenous metastases most commonly to lung, other bones, and the bone marrow. 3,4,11,13,15 Such tumors can also occur in the skull, spine, and brain as primary tumors or can be of metastatic origin. 3,4,15 Microscopically, the sheets of tumor cells are relatively monomorphous with a round to angular nucleus, a thin cytoplasmic margin, and irregular border.…”

“…3,4,11,13,15 These highly aggressive peripheral primitive neuroectodermal tumors (pPNETs) are histologically categorized as pediatric ''small round blue cell tumors.'' 3,4,11,13 As a group, pPNETs have been clearly separated from classical PNETs of the peripheral nervous system (PNS) (e.g., neuroblastoma, ganglioneuroblastoma, and ganglioneuroma) by differences in their clinical, pathologic, and molecular genetic profiles. [1][2][3][4]8,11,13,15 Currently the EFT group includes the intraosseous pPNET (or Ewing's sarcoma), the extraosseous pPNET and the thoracopulmonary Askin's tumor.…”

“…[1][2][3][4]8,11,13,15 Currently the EFT group includes the intraosseous pPNET (or Ewing's sarcoma), the extraosseous pPNET and the thoracopulmonary Askin's tumor. 3,4,13,15 The primary site of Ewing's sarcoma arises in the long tubular bones and pelvis with subsequent hematogenous metastases most commonly to lung, other bones, and the bone marrow. 3,4,11,13,15 Such tumors can also occur in the skull, spine, and brain as primary tumors or can be of metastatic origin.…”