Primary intradural Ewing’s sarcoma of the spine: a systematic review of the literature

Lu, Victor M.; Goyal, Anshit; Alvi, Mohammed Ali; Kerezoudis, Panagiotis; Haddock, Michael G.; Bydon, Mohamad

doi:10.1016/j.clineuro.2018.12.011

Cited by 23 publications

(36 citation statements)

References 46 publications

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“…Distant craniospinal axis failure is the predominant event in patients treated with focal radiotherapy. 2 The patient was treated according to the EURO EWING-99 protocol with 6 courses of vincristine, ifosfamide, doxorubicin, and etoposide (VIDE). Four weeks following the VIDE course, the patient started photon irradiation therapy against the entire brain and spinal cord with 36 Gy and a boost to the lumbar spine to a local total dose of 50.4 Gy.…”

Section: Oncologymentioning

confidence: 99%

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Intradural Extramedullary Ewing Sarcoma in an Adolescent Female

Kunwald¹,

Straszek²,

Bjarkam³

et al. 2022

AHMT

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A 15-year-old female experiencing lumbar pain without prior trauma. Despite being prescribed strong analgesics by her family physician, the symptoms intensified, and she was referred to diagnostic imaging. Magnetic resonance imaging revealed an intradural extramedullary tumor in the spinal canal, located behind the L3 corpora. The patient underwent neurosurgery with a complete resection of the tumor. The histopathologic examination revealed Ewing sarcoma by identifying EWSR1 gene in the extracted tissue. The patient was subsequently referred for extensive specialized oncological treatment including photon irradiation therapy and chemotherapy. Thirty-six months on from the initial diagnosis, the patient is doing well and suffers no sequelae apart from hypogonadism.

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Section: Oncologymentioning

confidence: 99%

“…Distant craniospinal axis failure is the predominant event in patients treated with focal radiotherapy. 2 …”

Section: Oncologymentioning

confidence: 99%

Intradural Extramedullary Ewing Sarcoma in an Adolescent Female

Kunwald¹,

Straszek²,

Bjarkam³

et al. 2022

AHMT

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“…Sebuah tinjauan sistematis mengenai kasus Primary Spinal Intradural Ewing Sarcoma memperlihatkan hingga pertengahan tahun 2018, hanya ada 9 kasus pada anak yang telah dipublikasi. 3 Paterakis dkk mengatakan walaupun jarang, ES intraduralekstramedula mereka memiliki tiga karakteristik penting yang membedakan mereka dari ekstraskeletal ES lainnya. Pertama, lokasi lesi yang melekat pada struktur medulla spinalis dan radiks sering reseksi tumor secara total menjadi sulit.…”

Section: Diskusiunclassified

“…Tinjauan sistematis dari kasus Primary Spinal Intradural ES anak dan dewasa memperlihatkan belum adanya kesamaan untuk dosis radiasi yang diberikan, dengan variasi antara 17.5 -59.4 Gy dalam fraksi konvensional. 3 Panduan dari NCCN untuk Ewing Sarcoma pada tulang merekomendasikan dosis radioterapi adjuvan minimal 45 Gy pasca reseksi komplit dan 55.8 Gy bila ada residu pasca operasi. 21 Sementara toleransi radiasi untuk medulla spinalis daerah thoraks adalah 45 Gy untuk mencegah timbulnya mielopati akibat radiasi.…”

Section: Diskusiunclassified

Primary Spinal Intradural Ewing Sarcoma pada Anak: Sebuah Laporan Kasus di Masa Pandemi COVID-19

Herdian

Aman

Gondhowiardjo

2020

Radioter Onkol Indones

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Ewing Sarcoma (ES) merupakan kanker tulang primer nomor dua paling umum pada pasien anak. Manifestasi ekstraskeletal dari Sarkoma Ewing pada anak sangat jarang, terutama di rongga intradural pada tulang belakang.Penulis melaporkan sebuah kasus yaitu seorang anak berusia 14 tahun yang dirujuk ke departemen kami dengan Sarkoma Ewing pada tulang vertebrae T11. Pasien sebelumnya mengeluh nyeri punggung bawah yang progresif disertai paraparesis dan kesulitan miksi. MRI spinal menunjukkan massa intradura ekstramedulla setinggi tulang vertebrae T11 tanpa perubahan patologis dari tulang vertebrae. Pasien menjalani laminektomi torakal dan memperoleh gross total resection. Diagnosis Sarkoma Ewing ditegakkan dari pemeriksaan histopatologi dan immunohistokimia. PET/CT pasca operasi menunjukkan tangkapan FDG minimal di intradura T11 tanpa gambaran metastasis. Keputusan dari pertemuan multidisiplin tumor board adalah kemoterapi adjuvan diikuti radioterapi lokal. Pasca kemoterapi, rencana pemberian radioterapi ditunda akibat pandemi COVID-19. Pasien dijadwalkan untuk kontrol rutin dan MRI spinal per 3 bulan.Literatur menunjukkan diagnosis primary spinal intradural Ewing Sarcoma ditegakkan melalui pemeriksaan immunohistokimia dan/atau profil molekuler dengan pemeriksaan penunjang menyeluruh untuk menyingkirkan kemungkinan primer di daerah lain. Terapi diberikan sesuai penatalaksanaan untuk sarkoma ewing pada tulang yaitu kombinasi operasi diikuti kemoterapi dan radioterapi. Peran radioterapi pada Ewing Sarcoma ekstraskeletal saat ini terbatas untuk kontrol lokal dan direkomendasikan pasca reseksi subtotal. Radioterapi adjuvan dapat dihindari pada masa pandemi COVID-19 bila terbukti tidak meningkatkan kesintasan.

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“…However, the impact of neuroaxis irradiation (cranio-spinal (CSI) or spinal irradiation (SI)) on local control and survival of patients with EwS in this location remains unclear. Published data are mostly limited to case reports or case series reporting patients with primary intraspinal EwS treated with local RT to macroscopic tumor manifestations [ 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 ].…”

Section: Introductionmentioning

confidence: 99%

The Role of Neuroaxis Irradiation in the Treatment of Intraspinal Ewing Sarcoma: A Review and Meta-Analysis

Troschel

Kröger

Siats

et al. 2022

Cancers

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The role of cranio-spinal irradiation (CSI) for primary extraosseous intraspinal Ewing sarcoma (EwS) remains unclear. Here, we evaluate clinical and survival outcomes in patients with primary intraspinal EwS treated with CSI as part of multimodal primary therapy regimens. We abstracted patient information, including details on treatment application, efficacy, and tolerance from the literature and our hospital database for a cohort of 24 primary intraspinal EwS patients treated with CSI. Median age was 25.5 years, median CSI dose was 36 Gy and mean boost dose was 12.8 Gy. Sixteen patients (66.7%) achieved complete radiological remission, another 5 patients demonstrated partial response and 1 patient showed no response to treatment. Compared to a cohort of patients treated with focal radiotherapy, CSI patients were more likely to have multifocal disease at time of diagnosis (p = 0.001) and intradural tumor location (p < 0.001). Despite over-representation of these unfavorable characteristics, there was no survival difference between groups (p = 0.58). While CSI shows promising results in the treatment of primary intraspinal EwS, treatment should be considered individually based on tumor and patient characteristics in the absence of prospective trials.

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Primary intradural Ewing’s sarcoma of the spine: a systematic review of the literature

Cited by 23 publications

References 46 publications

Intradural Extramedullary Ewing Sarcoma in an Adolescent Female

Intradural Extramedullary Ewing Sarcoma in an Adolescent Female

Primary Spinal Intradural Ewing Sarcoma pada Anak: Sebuah Laporan Kasus di Masa Pandemi COVID-19

The Role of Neuroaxis Irradiation in the Treatment of Intraspinal Ewing Sarcoma: A Review and Meta-Analysis

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