Primary intradural extraosseous Ewing’s sarcoma of the L3 nerve root: illustrative case

John D Arena Departments of Neurosurgery, and

Search for other papers by John D Arena in
Current site
jns
Google Scholar
PubMed
Close
 MD
,
Saurabh Sinha Departments of Neurosurgery, and

Search for other papers by Saurabh Sinha in
Current site
jns
Google Scholar
PubMed
Close
 MD
,
Connor Wathen Departments of Neurosurgery, and

Search for other papers by Connor Wathen in
Current site
jns
Google Scholar
PubMed
Close
 MD
,
Yohannes Ghenbot Departments of Neurosurgery, and

Search for other papers by Yohannes Ghenbot in
Current site
jns
Google Scholar
PubMed
Close
 MD
,
Paul J Zhang Pathology and Laboratory Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania

Search for other papers by Paul J Zhang in
Current site
jns
Google Scholar
PubMed
Close
 MD
, and
William C Welch Departments of Neurosurgery, and

Search for other papers by William C Welch in
Current site
jns
Google Scholar
PubMed
Close
 MD
Open access

BACKGROUND

Ewing’s sarcoma is an uncommon, aggressive malignancy that typically presents as an osseous lesion, most commonly in children and adolescents. Very rarely Ewing’s sarcoma can present as an intradural extramedullary mass mimicking more common tumors.

OBSERVATIONS

A 32-year-old female had a left L3 nerve root–associated lesion identified in the setting of recent-onset radiculopathy. Contrast-enhanced magnetic resonance imaging of the lumbar spine was favored to demonstrate a schwannoma or neurofibroma. Hemilaminectomy, facetectomy, and resection of the mass led to improved radiculopathy and a tissue diagnosis of Ewing’s sarcoma. Immediate referral to medical oncology facilitated expeditious initiation of adjuvant chemotherapy and radiation.

LESSONS

The differential diagnosis for newly identified nerve root–associated tumors should remain broad, including common benign pathologies and rare malignant entities. Tissue remains the gold standard for diagnosis, as preoperative imaging suggested a nerve sheath tumor. Malignant pathologies such as Ewing’s sarcoma must be considered, especially in the setting of rapidly progressive symptoms or interval growth on serial imaging. Early diagnosis allows for the timely initiation of comprehensive oncological care. Long-term multidisciplinary follow-up is necessary for the surveillance of disease progression.

ABBREVIATIONS

CT = computed tomography; FISH = fluorescence in situ hybridization; IE = ifosfamide and etoposide; MRI = magnetic resonance imaging; PIEES = primary intradural extraosseous Ewing’s sarcoma; POD = postoperative day; VAC = vincristine, doxorubicinand, and cyclophosphamide chemotherapy

BACKGROUND

Ewing’s sarcoma is an uncommon, aggressive malignancy that typically presents as an osseous lesion, most commonly in children and adolescents. Very rarely Ewing’s sarcoma can present as an intradural extramedullary mass mimicking more common tumors.

OBSERVATIONS

A 32-year-old female had a left L3 nerve root–associated lesion identified in the setting of recent-onset radiculopathy. Contrast-enhanced magnetic resonance imaging of the lumbar spine was favored to demonstrate a schwannoma or neurofibroma. Hemilaminectomy, facetectomy, and resection of the mass led to improved radiculopathy and a tissue diagnosis of Ewing’s sarcoma. Immediate referral to medical oncology facilitated expeditious initiation of adjuvant chemotherapy and radiation.

LESSONS

The differential diagnosis for newly identified nerve root–associated tumors should remain broad, including common benign pathologies and rare malignant entities. Tissue remains the gold standard for diagnosis, as preoperative imaging suggested a nerve sheath tumor. Malignant pathologies such as Ewing’s sarcoma must be considered, especially in the setting of rapidly progressive symptoms or interval growth on serial imaging. Early diagnosis allows for the timely initiation of comprehensive oncological care. Long-term multidisciplinary follow-up is necessary for the surveillance of disease progression.

ABBREVIATIONS

CT = computed tomography; FISH = fluorescence in situ hybridization; IE = ifosfamide and etoposide; MRI = magnetic resonance imaging; PIEES = primary intradural extraosseous Ewing’s sarcoma; POD = postoperative day; VAC = vincristine, doxorubicinand, and cyclophosphamide chemotherapy

Ewing’s sarcoma is a rare malignant cancer primarily affecting children and adolescents, with fewer than 500 cases reported annually in the United States.1 Histopathologically, Ewing’s sarcoma is characterized as a poorly differentiated, small round blue cell tumor and is most commonly identified as a primary bone malignancy.2 Less frequently, Ewing’s sarcoma can present as a primary soft tissue, or extraosseous, tumor.3 Ewing’s sarcoma can occur along the spinal column as an osseous or extraosseous tumor; however, primary intradural extraosseous Ewing’s sarcoma (PIEES) is an exceedingly rare entity, with few cases described in the literature.4–34 PIEES can mimic more commonly encountered intradural pathologies, such as schwannomas, neurofibromas, ependymomas, meningiomas, or metastases, but has different treatment paradigms, highlighting the importance of prompt recognition and tissue diagnosis.32 Herein, we describe the case of a 32-year-old female who presented with left lower-extremity radiculopathy and was found to have PIEES of the L3 nerve root.

Illustrative Case

A 32-year-old female with no significant past medical history presented with 4 months of left lower-extremity radicular pain and paresthesias that failed conservative management of physical therapy and oral steroids. She did not endorse weakness or bowel or bladder dysfunction. Outpatient noncontrast magnetic resonance imaging (MRI) of the lumbar spine demonstrated a 0.9 × 1.4 × 1.5 cm T1 hypointense and T2 hyperintense nodular mass involving the left L3 nerve root, extending into the neural foramen. She subsequently underwent contrast MRI of the lumbar spine 13 days later, redemonstrating the nodular lesion, which was found to be grossly unchanged in size and mildly contrast enhancing (Fig. 1). The radiological differential diagnosis favored schwannoma or neurofibroma.

FIG. 1
FIG. 1

Preoperative sagittal (A–C) and axial (D–F) MRI of the lumbar spine. A nodular mass associated with the left L3 nerve root was T1 hypointense (A and D), T2 hyperintense (C and F), and mildly contrast enhancing (B and E).

The patient was referred for neurosurgical consultation. On initial examination, she was found to have no focal motor weakness; however, she demonstrated decreased sensation to light touch and pinprick in the L3–4 distribution. Resection of the lesion was recommended for nerve root decompression and tissue diagnosis.

We performed a left-sided L3 hemilaminectomy and medial facetectomy. The nerve root was identified and appeared grossly expanded. An associated firm, rubbery tumor was appreciated, which appeared to grossly infiltrate the nerve root. Further considerable facetectomy was performed laterally to adequately access the borders of the lesion. The nerve root sleeve was sharply opened, and tumor was dissected off the nerve root using a combination of microsurgical technique and a CO2 laser, until no residual tumor was appreciated. The nerve root was stimulated at 5–8 mA before and after tumor resection, suggesting preserved nerve function. Closure was performed using standard technique.

Postoperatively, the patient noted nearly complete relief of her radicular symptoms, and no new neurological deficit was appreciated. MRI was performed on postoperative day (POD) 1, demonstrating post-surgical changes and interval resection of the L3 nerve root–associated lesion, without evidence of residual disease (Fig. 2A and B). The patient was discharged home on POD 1 after working with physical therapy.

FIG. 2
FIG. 2

Postoperative MRI of the lumbar spine. POD 1 postcontrast T1-weighted (A) and T2-weighted (B) MRI demonstrated post-surgical changes together with interval resection of the left L3 nerve root–associated mass. Surveillance MRI 26 months after surgery (C and D) demonstrated no evidence of local disease recurrence or the development of new malalignment.

Microscopic pathological assessment revealed monotonous, poorly differentiated, small round blue cells with infiltration of adjacent nerve tissue (Fig. 3A and B). No necrosis was appreciated, and the Ki-67 proliferation index was estimated at 15%. Tumor cells expressed cyclin D1, BCL2, and MIC-2 (Fig. 3C) with patchy S100 (Fig. 3D) but were negative for lymphoid markers. Fluorescence in situ hybridization (FISH) analysis revealed EWSR1 gene rearrangement, confirming a diagnosis of Ewing’s sarcoma.

FIG. 3
FIG. 3

Diagnostic pathology of L3 nerve root–associated Ewing’s sarcoma. A: Hematoxylin and eosin (H&E) staining demonstrated small round blue cell tumor with invasion of adjacent neural tissue. B: High-power H&E staining showed sheets of monotonous small round blue cells. C: Strong membranous expression of MIC-2 further supported the diagnosis of Ewing’s sarcoma. D: In contrast, patchy expression of S100 was seen. Original magnification ×5 (A), ×20 (B–D).

Care was established with medical oncology in an expedited manner. Chest computed tomography (CT) and whole-body positron emission tomography/CT were performed on POD 21 without any evidence of metastatic disease. Lumbar spine MRI with and without contrast at this time revealed the expected reactive postoperative changes but no local recurrence. Given the aggressive nature of the tumor and high likelihood of recurrence despite resection, chemotherapy was initiated on POD 35. The patient underwent three cycles of alternating vincristine, doxorubicin, and cyclophosphamide chemotherapy (VAC) and ifosfamide and etoposide (IE), followed by local radiotherapy and then another three cycles of alternating VAC and IE.

The patient is now more than 2 years out from her diagnosis and has tolerated systemic oncological treatments. She has experienced minimal intermittent low-back pain, which is not lifestyle limiting, as she has resumed work and daily exercise. She has been monitored with serial imaging. Chest CT and lumbar spine MRI 26 months after surgery (Fig. 2C and D) did not demonstrate any evidence of local disease recurrence or metastatic disease.

Patient Informed Consent

The necessary patient informed consent was obtained in this study.

Discussion

Observations

The above case report describes the clinical manifestation, surgical management, pathological diagnosis, and adjuvant chemo-radiotherapeutic management of PIEES of the L3 nerve root, an exceedingly rare presentation of an already rare malignancy. Our report adds to the limited number of such cases described in the literature to date.4–34 Our case demonstrates the challenges in distinguishing this unusual entity from other similar-appearing and significantly more common intradural pathologies. The implications of arriving at this unexpected diagnosis and the consequences surrounding postoperative management highlight the importance of obtaining tissue for definitive pathological assessment.

Lessons

Ewing’s sarcoma as a primary nerve root–associated tumor is a rare presentation of an already uncommon malignancy. Osseous Ewing’s sarcoma, extraosseous Ewing’s sarcoma, and peripheral primitive neuroectodermal tumor were initially considered to be distinct pathological entities; however, considerable histological and genetic similarities have led to the recognition of these tumors as closely related members of what has been deemed the “Ewing’s sarcoma family of tumors.”3 Ewing’s sarcoma is generally considered a malignancy of childhood and adolescence, and initial presentation can occur less frequently in the third and fourth decades of life. A highly malignant small round blue cell tumor, Ewing’s sarcoma’s pathogenesis is thought to be driven by chromosome 22 translocation involving the ESWR1 gene and the resultant oncogenic fusion protein.35 Identified histologically as a small round blue cell tumor, Ewing’s sarcoma is marked by high expression of MIC-2, which can be detected by immunohistochemistry (Fig. 3C), an approach commonly utilized in diagnostic pathology.36,37 Identification of the ESWR1 translocation by way of molecular genetic testing, such as FISH or reverse transcriptase–polymerase chain reaction, serves as a sensitive and specific approach for confirmation of the diagnosis of Ewing’s sarcoma.38

PIEES is quite rare, with a limited number of cases described in the literature. Preoperative imaging typically reveals an intradural extramedullary mass, with an associated differential diagnosis including far more common entities such as schwannoma, neurofibroma, ependymoma, meningioma, and metastatic neoplasm. Whereas spinal nerve sheath tumors typically demonstrate clear enhancement on postcontrast T1-weighted MRI sequences, studies with relatively muted enhancement, as was appreciated in our case, suggest the possibility of an alternative rarer diagnosis. Given the generally low index of suspicion, however, the diagnosis of intradural Ewing’s sarcoma is typically not considered until pathology results are returned from tumor resection or biopsy.

Ewing’s sarcoma is particularly aggressive, with high rates of local and metastatic recurrence. The presence or absence of identifiable metastatic disease serves as the principal prognostic factor, with 5-year survival rates approximately twice as high if no overt metastatic disease is present at the time of initial diagnosis.39 Even though metastatic disease may not be initially identifiable, local therapy alone is considered insufficient, because such approaches often fail to prevent the development of metastases. Therefore, early systemic chemotherapy, followed by locally targeted interventions such as surgery and/or radiation, has become the mainstay of treatment.40 The generally preferred approach for treating extraosseous Ewing’s sarcoma is neoadjuvant chemotherapy followed by en bloc resection of tumor with the maintenance of clear margins.41 Such an approach is rarely strictly followed in the treatment of PIEES. Proximity to the spinal cord and nerve roots in PIESS limits the feasibility of true en bloc resection. Furthermore, given the initial low index of suspicion, diagnosis is typically not obtained until after resection.32 Resultantly, patients necessarily undergo adjuvant induction of chemotherapy, with or without radiotherapy.32 As treatment strategies for Ewing’s sarcoma have been refined in recent decades, prognosis has continued to improve. Contemporary paradigms have led to 5-year overall survival rates greater than 70% for patients initially diagnosed with only local disease.41–44

Our patient is more than 2 years out from her initial diagnosis and, fortunately, has not demonstrated any evidence of local disease recurrence or distant metastasis. She will continue to require close multidisciplinary surveillance. Beyond primary oncological follow-up, she warrants structural monitoring of her lumbar spine. Surgical access to her lesion was accomplished through significant unilateral facetectomy, potentially contributing to the development of instability at L3–4. Thus far, the patient has only experienced occasional mild back pain, and serial follow-up lumbar MRI has not demonstrated the development of progressive malalignment or instability. Only in her fourth decade of life, however, she warrants long-term neurosurgical follow-up.

Our case adds to the limited number reported in the literature to date. The surprising diagnosis of Ewing’s sarcoma, only elucidated following resection, is typical for these initially benign-appearing lesions. Cases such as ours highlight the importance of obtaining a tissue diagnosis to facilitate prompt adjuvant treatment for these malignant tumors. Ewing’s sarcoma, although rare, warrants consideration in the initial workup of intradural extramedullary lesions. Optimizing outcomes for patients with PIEES necessitates prompt recognition of pathology, collaboration with colleagues in oncology, and resection with an emphasis on maximal safe marginal excision.

Author Contributions

Conception and design: Arena, Sinha, Welch. Acquisition of data: Arena, Zhang, Welch. Analysis and interpretation of data: Arena, Ghenbot, Zhang, Welch. Drafting the article: Arena, Wathen. Critically revising the article: Arena, Wathen, Ghenbot, Welch. Reviewed submitted version of manuscript: Arena, Sinha, Wathen, Ghenbot. Approved the final version of the manuscript on behalf of all authors: Arena. Administrative/technical/material support: Arena. Study supervision: Welch.

References

  • 1

    Arndt CA, Rose PS, Folpe AL, Laack NN Common musculoskeletal tumors of childhood and adolescence. Mayo Clin Proc. 2012;87(5):475487.

  • 2

    de Alava E, Gerald WL Molecular biology of the Ewing’s sarcoma/primitive neuroectodermal tumor family. J Clin Oncol. 2000;18(1):204213.

  • 3

    Grier HE The Ewing family of tumors. Ewing’s sarcoma and primitive neuroectodermal tumors. Pediatr Clin North Am. 1997;44(4):9911004.

  • 4

    Hisaoka M, Hashimoto H, Murao T Peripheral primitive neuroectodermal tumour with ganglioneuroma-like areas arising in the cauda equina. Virchows Arch. 1997;431(5):365369.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 5

    Isotalo PA, Agbi C, Davidson B, Girard A, Verma S, Robertson SJ Primary primitive neuroectodermal tumor of the cauda equina. Hum Pathol. 2000;31(8):9991001.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 6

    Harimaya K, Oda Y, Matsuda S, Tanaka K, Chuman H, Iwamoto Y Primitive neuroectodermal tumor and extraskeletal Ewing sarcoma arising primarily around the spinal column: report of four cases and a review of the literature. Spine (Phila Pa 1976). 2003;28(19):E408E412.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 7

    Uesaka T, Amano T, Inamura T, et al. Intradural, extramedullary spinal Ewing’s sarcoma in childhood. J Clin Neurosci. 2003;10(1):122125.

  • 8

    Fabre E, Guillevin R, Chretien F, Le Guerinel C, Duffau H Peripheral primitive neuroectodermal tumor of the cauda equina in an elderly patient. Case report. J Neurosurg Spine. 2006;5(1):6871.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 9

    Mobley BC, Roulston D, Shah GV, Bijwaard KE, McKeever PE Peripheral primitive neuroectodermal tumor/Ewing’s sarcoma of the craniospinal vault: case reports and review. Hum Pathol. 2006;37(7):845853.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 10

    Haresh KP, Chinikkatti SK, Prabhakar R, et al. A rare case of intradural extramedullary Ewing’s sarcoma with skip metastasis in the spine. Spinal Cord. 2008;46(8):582584.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 11

    Kim SW, Shin H Primary intradural extraosseous Ewing’s sarcoma. J Korean Neurosurg Soc. 2009;45(3):179181.

  • 12

    Klimo P Jr, Codd PJ, Grier H, Goumnerova LC Primary pediatric intraspinal sarcomas. Report of 3 cases. J Neurosurg Pediatr. 2009;4(3):222229.

  • 13

    Muzzafar S, Rhines LD, Bruner J, Schellingerhout D Intradural extramedullary Ewing tumor of the lumbar spine. Radiol Case Rep. 2015;5(4):421.

  • 14

    Vincentelli F, Caruso G, Figarella-Branger D Primary intradural Ewing’s sarcoma of the cauda equina presenting with acute bleeding. Acta Neurochir (Wien). 2010;152(3):563564.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 15

    Duan XH, Ban XH, Liu B, et al. Intraspinal primitive neuroectodermal tumor: imaging findings in six cases. Eur J Radiol. 2011;80(2):426431.

  • 16

    Ellis JA, Rothrock RJ, Moise G, et al. Primitive neuroectodermal tumors of the spine: a comprehensive review with illustrative clinical cases. Neurosurg Focus. 2011;30(1):E1.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 17

    Karikari IO, Mehta AI, Nimjee S, et al. Primary intradural extraosseous Ewing sarcoma of the spine: case report and literature review. Neurosurgery. 2011;69(4):E995E999.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 18

    Mateen FJ, Nassar A, Bardia A, et al. Spinal intradural extraosseous Ewing’s sarcoma. Rare Tumors. 2011;3(1):e7.

  • 19

    Yan Y, Xu T, Chen J, Hu G, Lu Y Intraspinal Ewing’s sarcoma/primitive neuroectodermal tumors. J Clin Neurosci. 2011;18(5):601606.

  • 20

    Bazzocchi A, Bacci A, Serchi E, Salerno A, Salizzoni E, Leonardi M Intradural extramedullary Ewing’s sarcoma. Recurrence with acute clinical presentation and literature review. Neuroradiol J. 2013;26(4):476481.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 21

    Khalatbari MR, Jalaeikhoo H, Moharamzad Y Primary intradural extraosseous Ewing’s sarcoma of the lumbar spine presenting with acute bleeding. Br J Neurosurg. 2013;27(6):840841.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 22

    Pancucci G, Simal-Julian JA, Plaza-Ramirez E, García-Marcos R, Mayordomo-Aranda E, Botella-Asunción C Primary extraosseous intradural spinal Ewing’s sarcoma: report of two cases. Acta Neurochir (Wien). 2013;155(7):12291234.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 23

    Lozupone E, Martucci M, Rigante L, Gaudino S, Di Lella GM, Colosimo C Magnetic resonance image findings of primary intradural Ewing sarcoma of the cauda equina: case report and review of the literature. Spine J. 2014;14(4):e7e11.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 24

    Mardekian SK, Gandhe A, Miettinen M, Pack S, Curtis MT, Abdullaev Z Two cases of spinal, extraosseous, intradural Ewing’s sarcoma/peripheral neuroectodermal tumor: radiologic, pathologic, and molecular analysis. J Clin Imaging Sci. 2014;4:6.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 25

    Zhao M, Zhang B, Liang F, Zhang J Primary spinal intradural extraskeletal Ewing sarcoma mimicking a giant nerve sheath tumor: case report and review of the literature. Int J Clin Exp Pathol. 2014;7(12):90819085.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 26

    Bostelmann R, Leimert M, Steiger HJ, Gierga K, Petridis AK The importance of surgery as part of multimodal therapy in rapid progressive primary extraosseous Ewing sarcoma of the cervical intra- and epidural space. Clin Pract. 2016;6(4):897.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 27

    Chihak MA, Ahmed SK, Lachance DH, Nageswara Rao AA, Laack NN Patterns of failure and optimal radiotherapy target volumes in primary intradural extramedullary Ewing sarcoma. Acta Oncol. 2016;55(8):10571061.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 28

    Kartal A, Akatlı A Primary intradural extraosseous Ewing’s sarcoma in a young child. Childs Nerv Syst. 2016;32(3):409410.

  • 29

    Paterakis K, Brotis A, Tasiou A, Kotoula V, Kapsalaki E, Vlychou M Intradural extramedullary Ewing’s sarcoma: a case report and review of the literature. Neurol Neurochir Pol. 2017;51(1):106110.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 30

    Takami H, Kumar R, Brown DA, Krauss WE Histologic features and prognosis of spinal intradural extramedullary Ewing sarcoma: case report, literature review, and analysis of prognosis. World Neurosurg. 2018;115:448452.e2.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 31

    Lu VM, Goyal A, Alvi MA, Kerezoudis P, Haddock MG, Bydon M Primary intradural Ewing’s sarcoma of the spine: a systematic review of the literature. Clin Neurol Neurosurg. 2019;177:1219.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 32

    Amelot A, Peyre M, Mokhtari K, et al. Primary extraosseous spinal Ewing sarcomas: should we be more aware about diagnosis? Spine (Phila Pa 1976). 2021;46(5):313321.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 33

    Pu F, Liu J, Zhang Z, Guo T, Shao Z Primary intradural extramedullary extraosseous Ewing’s sarcoma/peripheral primitive neuroectodermal tumor (PIEES/PNET) of the thoracolumbar spine: a case report and literature review. Open Med (Wars). 2021;16(1):15911596.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 34

    Kunwald M, Straszek CL, Bjarkam CR, et al. Intradural extramedullary Ewing sarcoma in an adolescent female. Adolesc Health Med Ther. 2022;13:3943.

  • 35

    Delattre O, Zucman J, Melot T, et al. The Ewing family of tumors—a subgroup of small-round-cell tumors defined by specific chimeric transcripts. N Engl J Med. 1994;331(5):294299.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 36

    Ambros IM, Ambros PF, Strehl S, Kovar H, Gadner H, Salzer-Kuntschik M MIC2 is a specific marker for Ewing’s sarcoma and peripheral primitive neuroectodermal tumors. Evidence for a common histogenesis of Ewing’s sarcoma and peripheral primitive neuroectodermal tumors from MIC2 expression and specific chromosome aberration. Cancer. 1991;67(7):18861893.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 37

    Zhang PJ, Barcos M, Stewart CC, Block AW, Sait S, Brooks JJ Immunoreactivity of MIC2 (CD99) in acute myelogenous leukemia and related diseases. Mod Pathol. 2000;13(4):452458.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 38

    Italiano A, Di Mauro I, Rapp J, et al. Clinical effect of molecular methods in sarcoma diagnosis (GENSARC): a prospective, multicentre, observational study. Lancet Oncol. 2016;17(4):532538.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 39

    Cotterill SJ, Ahrens S, Paulussen M, et al. Prognostic factors in Ewing’s tumor of bone: analysis of 975 patients from the European Intergroup Cooperative Ewing’s Sarcoma Study Group. J Clin Oncol. 2000;18(17):31083114.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 40

    Grier HE, Krailo MD, Tarbell NJ, et al. Addition of ifosfamide and etoposide to standard chemotherapy for Ewing’s sarcoma and primitive neuroectodermal tumor of bone. N Engl J Med. 2003;348(8):694701.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 41

    Charest-Morin R, Dirks MS, Patel S, et al. Ewing sarcoma of the spine: prognostic variables for survival and local control in surgically treated patients. Spine (Phila Pa 1976). 2018;43(9):622629.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 42

    Obata H, Ueda T, Kawai A, et al. Clinical outcome of patients with Ewing sarcoma family of tumors of bone in Japan: the Japanese Musculoskeletal Oncology Group cooperative study. Cancer. 2007;109(4):767775.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 43

    Rodríguez-Galindo C, Liu T, Krasin MJ, et al. Analysis of prognostic factors in ewing sarcoma family of tumors: review of St. Jude Children’s Research Hospital studies. Cancer. 2007;110(2):375384.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 44

    Applebaum MA, Worch J, Matthay KK, et al. Clinical features and outcomes in patients with extraskeletal Ewing sarcoma. Cancer. 2011;117(13):30273032.

  • Collapse
  • Expand
  • FIG. 1

    Preoperative sagittal (A–C) and axial (D–F) MRI of the lumbar spine. A nodular mass associated with the left L3 nerve root was T1 hypointense (A and D), T2 hyperintense (C and F), and mildly contrast enhancing (B and E).

  • FIG. 2

    Postoperative MRI of the lumbar spine. POD 1 postcontrast T1-weighted (A) and T2-weighted (B) MRI demonstrated post-surgical changes together with interval resection of the left L3 nerve root–associated mass. Surveillance MRI 26 months after surgery (C and D) demonstrated no evidence of local disease recurrence or the development of new malalignment.

  • FIG. 3

    Diagnostic pathology of L3 nerve root–associated Ewing’s sarcoma. A: Hematoxylin and eosin (H&E) staining demonstrated small round blue cell tumor with invasion of adjacent neural tissue. B: High-power H&E staining showed sheets of monotonous small round blue cells. C: Strong membranous expression of MIC-2 further supported the diagnosis of Ewing’s sarcoma. D: In contrast, patchy expression of S100 was seen. Original magnification ×5 (A), ×20 (B–D).

  • 1

    Arndt CA, Rose PS, Folpe AL, Laack NN Common musculoskeletal tumors of childhood and adolescence. Mayo Clin Proc. 2012;87(5):475487.

  • 2

    de Alava E, Gerald WL Molecular biology of the Ewing’s sarcoma/primitive neuroectodermal tumor family. J Clin Oncol. 2000;18(1):204213.

  • 3

    Grier HE The Ewing family of tumors. Ewing’s sarcoma and primitive neuroectodermal tumors. Pediatr Clin North Am. 1997;44(4):9911004.

  • 4

    Hisaoka M, Hashimoto H, Murao T Peripheral primitive neuroectodermal tumour with ganglioneuroma-like areas arising in the cauda equina. Virchows Arch. 1997;431(5):365369.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 5

    Isotalo PA, Agbi C, Davidson B, Girard A, Verma S, Robertson SJ Primary primitive neuroectodermal tumor of the cauda equina. Hum Pathol. 2000;31(8):9991001.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 6

    Harimaya K, Oda Y, Matsuda S, Tanaka K, Chuman H, Iwamoto Y Primitive neuroectodermal tumor and extraskeletal Ewing sarcoma arising primarily around the spinal column: report of four cases and a review of the literature. Spine (Phila Pa 1976). 2003;28(19):E408E412.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 7

    Uesaka T, Amano T, Inamura T, et al. Intradural, extramedullary spinal Ewing’s sarcoma in childhood. J Clin Neurosci. 2003;10(1):122125.

  • 8

    Fabre E, Guillevin R, Chretien F, Le Guerinel C, Duffau H Peripheral primitive neuroectodermal tumor of the cauda equina in an elderly patient. Case report. J Neurosurg Spine. 2006;5(1):6871.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 9

    Mobley BC, Roulston D, Shah GV, Bijwaard KE, McKeever PE Peripheral primitive neuroectodermal tumor/Ewing’s sarcoma of the craniospinal vault: case reports and review. Hum Pathol. 2006;37(7):845853.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 10

    Haresh KP, Chinikkatti SK, Prabhakar R, et al. A rare case of intradural extramedullary Ewing’s sarcoma with skip metastasis in the spine. Spinal Cord. 2008;46(8):582584.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 11

    Kim SW, Shin H Primary intradural extraosseous Ewing’s sarcoma. J Korean Neurosurg Soc. 2009;45(3):179181.

  • 12

    Klimo P Jr, Codd PJ, Grier H, Goumnerova LC Primary pediatric intraspinal sarcomas. Report of 3 cases. J Neurosurg Pediatr. 2009;4(3):222229.

  • 13

    Muzzafar S, Rhines LD, Bruner J, Schellingerhout D Intradural extramedullary Ewing tumor of the lumbar spine. Radiol Case Rep. 2015;5(4):421.

  • 14

    Vincentelli F, Caruso G, Figarella-Branger D Primary intradural Ewing’s sarcoma of the cauda equina presenting with acute bleeding. Acta Neurochir (Wien). 2010;152(3):563564.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 15

    Duan XH, Ban XH, Liu B, et al. Intraspinal primitive neuroectodermal tumor: imaging findings in six cases. Eur J Radiol. 2011;80(2):426431.

  • 16

    Ellis JA, Rothrock RJ, Moise G, et al. Primitive neuroectodermal tumors of the spine: a comprehensive review with illustrative clinical cases. Neurosurg Focus. 2011;30(1):E1.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 17

    Karikari IO, Mehta AI, Nimjee S, et al. Primary intradural extraosseous Ewing sarcoma of the spine: case report and literature review. Neurosurgery. 2011;69(4):E995E999.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 18

    Mateen FJ, Nassar A, Bardia A, et al. Spinal intradural extraosseous Ewing’s sarcoma. Rare Tumors. 2011;3(1):e7.

  • 19

    Yan Y, Xu T, Chen J, Hu G, Lu Y Intraspinal Ewing’s sarcoma/primitive neuroectodermal tumors. J Clin Neurosci. 2011;18(5):601606.

  • 20

    Bazzocchi A, Bacci A, Serchi E, Salerno A, Salizzoni E, Leonardi M Intradural extramedullary Ewing’s sarcoma. Recurrence with acute clinical presentation and literature review. Neuroradiol J. 2013;26(4):476481.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 21

    Khalatbari MR, Jalaeikhoo H, Moharamzad Y Primary intradural extraosseous Ewing’s sarcoma of the lumbar spine presenting with acute bleeding. Br J Neurosurg. 2013;27(6):840841.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 22

    Pancucci G, Simal-Julian JA, Plaza-Ramirez E, García-Marcos R, Mayordomo-Aranda E, Botella-Asunción C Primary extraosseous intradural spinal Ewing’s sarcoma: report of two cases. Acta Neurochir (Wien). 2013;155(7):12291234.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 23

    Lozupone E, Martucci M, Rigante L, Gaudino S, Di Lella GM, Colosimo C Magnetic resonance image findings of primary intradural Ewing sarcoma of the cauda equina: case report and review of the literature. Spine J. 2014;14(4):e7e11.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 24

    Mardekian SK, Gandhe A, Miettinen M, Pack S, Curtis MT, Abdullaev Z Two cases of spinal, extraosseous, intradural Ewing’s sarcoma/peripheral neuroectodermal tumor: radiologic, pathologic, and molecular analysis. J Clin Imaging Sci. 2014;4:6.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 25

    Zhao M, Zhang B, Liang F, Zhang J Primary spinal intradural extraskeletal Ewing sarcoma mimicking a giant nerve sheath tumor: case report and review of the literature. Int J Clin Exp Pathol. 2014;7(12):90819085.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 26

    Bostelmann R, Leimert M, Steiger HJ, Gierga K, Petridis AK The importance of surgery as part of multimodal therapy in rapid progressive primary extraosseous Ewing sarcoma of the cervical intra- and epidural space. Clin Pract. 2016;6(4):897.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 27

    Chihak MA, Ahmed SK, Lachance DH, Nageswara Rao AA, Laack NN Patterns of failure and optimal radiotherapy target volumes in primary intradural extramedullary Ewing sarcoma. Acta Oncol. 2016;55(8):10571061.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 28

    Kartal A, Akatlı A Primary intradural extraosseous Ewing’s sarcoma in a young child. Childs Nerv Syst. 2016;32(3):409410.

  • 29

    Paterakis K, Brotis A, Tasiou A, Kotoula V, Kapsalaki E, Vlychou M Intradural extramedullary Ewing’s sarcoma: a case report and review of the literature. Neurol Neurochir Pol. 2017;51(1):106110.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 30

    Takami H, Kumar R, Brown DA, Krauss WE Histologic features and prognosis of spinal intradural extramedullary Ewing sarcoma: case report, literature review, and analysis of prognosis. World Neurosurg. 2018;115:448452.e2.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 31

    Lu VM, Goyal A, Alvi MA, Kerezoudis P, Haddock MG, Bydon M Primary intradural Ewing’s sarcoma of the spine: a systematic review of the literature. Clin Neurol Neurosurg. 2019;177:1219.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 32

    Amelot A, Peyre M, Mokhtari K, et al. Primary extraosseous spinal Ewing sarcomas: should we be more aware about diagnosis? Spine (Phila Pa 1976). 2021;46(5):313321.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 33

    Pu F, Liu J, Zhang Z, Guo T, Shao Z Primary intradural extramedullary extraosseous Ewing’s sarcoma/peripheral primitive neuroectodermal tumor (PIEES/PNET) of the thoracolumbar spine: a case report and literature review. Open Med (Wars). 2021;16(1):15911596.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 34

    Kunwald M, Straszek CL, Bjarkam CR, et al. Intradural extramedullary Ewing sarcoma in an adolescent female. Adolesc Health Med Ther. 2022;13:3943.

  • 35

    Delattre O, Zucman J, Melot T, et al. The Ewing family of tumors—a subgroup of small-round-cell tumors defined by specific chimeric transcripts. N Engl J Med. 1994;331(5):294299.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 36

    Ambros IM, Ambros PF, Strehl S, Kovar H, Gadner H, Salzer-Kuntschik M MIC2 is a specific marker for Ewing’s sarcoma and peripheral primitive neuroectodermal tumors. Evidence for a common histogenesis of Ewing’s sarcoma and peripheral primitive neuroectodermal tumors from MIC2 expression and specific chromosome aberration. Cancer. 1991;67(7):18861893.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 37

    Zhang PJ, Barcos M, Stewart CC, Block AW, Sait S, Brooks JJ Immunoreactivity of MIC2 (CD99) in acute myelogenous leukemia and related diseases. Mod Pathol. 2000;13(4):452458.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 38

    Italiano A, Di Mauro I, Rapp J, et al. Clinical effect of molecular methods in sarcoma diagnosis (GENSARC): a prospective, multicentre, observational study. Lancet Oncol. 2016;17(4):532538.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 39

    Cotterill SJ, Ahrens S, Paulussen M, et al. Prognostic factors in Ewing’s tumor of bone: analysis of 975 patients from the European Intergroup Cooperative Ewing’s Sarcoma Study Group. J Clin Oncol. 2000;18(17):31083114.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 40

    Grier HE, Krailo MD, Tarbell NJ, et al. Addition of ifosfamide and etoposide to standard chemotherapy for Ewing’s sarcoma and primitive neuroectodermal tumor of bone. N Engl J Med. 2003;348(8):694701.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 41

    Charest-Morin R, Dirks MS, Patel S, et al. Ewing sarcoma of the spine: prognostic variables for survival and local control in surgically treated patients. Spine (Phila Pa 1976). 2018;43(9):622629.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 42

    Obata H, Ueda T, Kawai A, et al. Clinical outcome of patients with Ewing sarcoma family of tumors of bone in Japan: the Japanese Musculoskeletal Oncology Group cooperative study. Cancer. 2007;109(4):767775.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 43

    Rodríguez-Galindo C, Liu T, Krasin MJ, et al. Analysis of prognostic factors in ewing sarcoma family of tumors: review of St. Jude Children’s Research Hospital studies. Cancer. 2007;110(2):375384.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 44

    Applebaum MA, Worch J, Matthay KK, et al. Clinical features and outcomes in patients with extraskeletal Ewing sarcoma. Cancer. 2011;117(13):30273032.

Metrics

All Time Past Year Past 30 Days
Abstract Views 0 0 0
Full Text Views 366 366 68
PDF Downloads 256 257 27
EPUB Downloads 0 0 0