RARE-30. A RARE CASE OF PRIMARY EWING’S SARCOMA OF THE CERVICAL SPINE

Abstract Ewing sarcoma family of tumors predominantly affect the pediatric population in the long bones of the extremities or the pelvis, and only 8% of cases arise within the spine. Primary Ewing’s sarcoma of the cervical spine is extremely rare and less than 30 cases have been reported in the literature thus far. Here we present a case of primary Ewing’s sarcoma of the cervical spine in a 28-year-old female who presented with a three-month history of neck pain and right arm radiculopathy. MRI revealed a homogeneously contrast enhancing, eccentric mass with dural tail at C2-C7. After undergoing a hemilaminectomy, histopathology confirmed extraosseous Ewing’s sarcoma with CD99 positivity. A comprehensive systemic and neuraxis work-up ruled out overt metastasis. We extrapolated data from children’s cooperative group studies and IESS-II clinical trial to formulate a three phase treatment protocol as described below. To date, patient is in remission with no evidence of any residual disease in the cervical spine. In conclusion, although Primary Ewing’s sarcoma of the cervical spine is extremely rare it should be considered a differential diagnosis in patients with neck pain and a spinal mass under the age of thirty. Less than 25% of EFT’s present with overt metastasis and almost all have subclinical metastatic disease at the time of diagnosis, therefore, a comprehensive evaluation and systemic chemotherapy is recommended. We recommend a multidisciplinary approach of surgical decompression to preserve neurological functions, followed by compressed chemotherapy regimens, reevaluation for local treatment, and adjuvant chemotherapy.

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Neoadjuvant Chemotherapy for Localized Ewing's Sarcoma of the Extremities: Preliminary Results of a Protocol Which uses Surgery (Alone or Followed by Radiotherapy) for Local Control

Tumori Journal ◽

10.1177/030089168907500511 ◽

1989 ◽

Vol 75 (5) ◽

pp. 456-462 ◽

Cited By ~ 4

Author(s):

Gaetano Bacci ◽

Dante Dallari ◽

Douglas McDonald ◽

Maddalena Avella ◽

Aldo Toni ◽

...

Keyword(s):

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Local Treatment ◽

Local Therapy ◽

Conservative Surgery ◽

Additional Chemotherapy ◽

Radiotherapy Alone ◽

One Year ◽

Almost All

From June 1983 to December 1985, thirty-eight paients with localized Ewing's sarcoma of the extremities were treated with a protocol that consisted of an initial nine week period of polychemotherapy (vincristine, adriamycin and cyclophosphamide) followed by local therapy and additional chemotherapy (vincristine, adriamycin, cyclophosphamide and dactino-mycln) for one year. As local treatment all patients were offered surgery; thirty-two accepted and six refused. These six patients were locally treated with radiotherapy alone (50 Gy). In the remaining patients an amputation was performed in one case and a resection in thirty-one. In resected patients when a wide margin was achieved (24 cases) no further local treatment was performed; when it was marginal (5 cases) or intralesional (2 cases) radiotherapy at lower doses (40 Gy) followed. At mean follow-up of thirty-seven months the percentage of continuously disease-free patients was 50 % for those treated with radiotherapy, 76 % with surgery, and 85 % with surgery and radiotherapy. Eight patients developed metastatic disease and two patients had local recurrence and metastases. The local recurrences were seen in one patient locally treated with surgery and in one locally treated with radiotherapy. Nine major local complications were observed: three in patients treated with radiotherapy, five in patients treated with surgery, and one in a patient treated with surgery and radiotherapy. These results indicate that after induction chemotherapy conservative surgery is possible in almost all cases of Ewing's sarcoma of the extremities and that such treatment is better than radiotherapy alone as local therapy.

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Role of surgery in local treatment of Ewing's sarcoma of the extremities in patients undergoing adjuvant and neoadjuvant chemotherapy

Oncology Reports ◽

10.3892/or.11.1.111 ◽

2004 ◽

Author(s):

Gaetano Bacci ◽

Stefano Ferrari ◽

Alessandra Longhi ◽

Davide Donati ◽

Enza Barbieri ◽

...

Keyword(s):

Neoadjuvant Chemotherapy ◽

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Local Treatment

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Ewing's tumors with primary lung metastases: survival analysis of 114 (European Intergroup) Cooperative Ewing's Sarcoma Studies patients.

Journal of Clinical Oncology ◽

10.1200/jco.1998.16.9.3044 ◽

1998 ◽

Vol 16 (9) ◽

pp. 3044-3052 ◽

Cited By ~ 170

Author(s):

M Paulussen ◽

S Ahrens ◽

A W Craft ◽

J Dunst ◽

B Fröhlich ◽

...

Keyword(s):

Prognostic Factors ◽

Primary Tumor ◽

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Lung Metastases ◽

Local Treatment ◽

Poor Response ◽

Chemotherapy Response ◽

Treatment Intensification ◽

Lung Irradiation

PURPOSE To analyze event-free survival (EFS) and prognostic factors in patients who present with Ewing's tumors (ET) of bone and synchronous pulmonary and/or pleural metastases (ppm). PATIENTS AND METHODS Of 1,270 patients (pts) registered at the continental office of the German/European Intergroup Cooperative Ewing's Sarcoma Studies (CESS81, CESS86, EICESS92), 114 were diagnosed ET with ppm. Patients underwent neoadjuvant therapy and local treatment of the primary tumor. Whole-lung irradiation 15 to 18 Gy was applied to 75 ppm-pts. EFS and 95% confidence intervals (CIs) were estimated according to the Kaplan-Meier method, and prognostic factors were analyzed by log-rank tests and Cox and logistic regression procedures. RESULTS On November 1, 1997, at a median time under study of 5.9 years, the 5-year EFS was 0.36 (95% CI, 0.26 to 0.46) and the 10-year EFS was 0.30 (95% CI, 0.19 to 0.41). Thirty-seven of 59 (63%) first relapses involved lung and/or pleura, and the lungs were the only site of relapse in 26 of 59 (44%) ppm-pts. Risk factors identified in univariate and multivariate tests were poor response of the primary tumor toward chemotherapy, metastatic lesions in both lungs, and treatment without additional lung irradiation. CONCLUSION Chemotherapy response of the primary tumor is a prognostic factor in patients with ET with ppm. Strategies of treatment intensification warrant further evaluation.

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Detection of circulating tumor cells in patients with Ewing's sarcoma and peripheral primitive neuroectodermal tumor.

Journal of Clinical Oncology ◽

10.1200/jco.1997.15.2.583 ◽

1997 ◽

Vol 15 (2) ◽

pp. 583-588 ◽

Cited By ~ 86

Author(s):

D C West ◽

H E Grier ◽

M M Swallow ◽

G D Demetri ◽

L Granowetter ◽

...

Keyword(s):

Bone Marrow ◽

Tumor Cells ◽

Primitive Neuroectodermal Tumor ◽

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Residual Disease ◽

Neuroectodermal Tumor ◽

Peripheral Primitive Neuroectodermal Tumor ◽

Rt Pcr ◽

Relapsed Disease

PURPOSE To determine the feasibility of detecting Ewing's sarcoma (ES) or peripheral primitive neuroectodermal tumor (PNET) through a reverse-transcriptase polymerase chain reaction (RT-PCR) of the t(11;22)(q24;q12) fusion transcript in blood and bone marrow samples from patients with these neoplasms. PATIENTS AND METHODS Peripheral-blood (PB) and/or bone marrow aspirate (BM) samples were obtained from 28 patients with ES or PNET at initial presentation or at relapse. Patients were divided into two groups: newly diagnosed patients with nonmetastatic disease and those with metastatic/relapsed disease. RNA was extracted from fractionated BM and PB samples, and RT-PCR was performed for the EWS/HumFLI1 fusion mRNA was transcribed across the t(11;22) breakpoint. RESULTS Among the 16 patients with nonmetastatic disease, three of 16 were RT-PCR positive for EWS/HumFLI1 RNA in BM and three of 10 were positive in PB. The total number of nonmetastatic patients who were positive in either PB or BM was four of 16 (25%). Among patients with metastatic/relapsed disease, two of six were positive in BM and five of 10 were positive in PB. The total fraction of patients with metastatic/relapsed disease that was positive in either BM or PB was six of 12 (50%). CONCLUSION In this study, we show that it is possible to amplify the EWS/HumFLI1 RNA by RT-PCR from the BM and PB of a subset of patients with both nonmetastatic and metastatic ES or PNET, which implies that occult tumor cells are present at these sites. The true biologic and clinical meaning of this information is unknown. However, it does suggest a possible application of RT-PCR for the monitoring of residual disease in patients who are undergoing therapy for ES or PNET. This approach may permit early identification of patients who may benefit from alternative therapy or who may be spared possible overtreatment.

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Treatment of Patients Aged over 50 Years with Non-Osseous Ewing's Sarcoma Family Tumors: Five Cases and Review of Literature

Tumori Journal ◽

10.1177/030089160809400606 ◽

2008 ◽

Vol 94 (6) ◽

pp. 809-812 ◽

Cited By ~ 2

Author(s):

Gil Bar-Sela ◽

Avivit Peer ◽

Shelly Rothschild ◽

Nissim Haim

Keyword(s):

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Pediatric Population ◽

Infectious Complications ◽

Treatment Modalities ◽

Review Of Literature ◽

Poor Prognostic Factor ◽

Treatment Delays ◽

Chemotherapy Protocol ◽

Dose Reductions

Background Most clinical trials on Ewing's sarcoma family of tumors include pediatric and adolescent populations, whereas clinical data on older patients are limited. Patients and Methods We report on 5 patients older than 50 years with a tumor of the Ewing's sarcoma family treated recently in our department. Results Myelosuppression and infectious complications were the main toxicity encountered. Major dose reductions and/or treatment delays were required in all 5 patients. One patient died of septic shock. Complete remission was achieved in the remaining 4 patients with the addition of different treatment modalities. One patient had lung metastasis 3 years after starting chemotherapy, and 3 patients have remained without evidence of recurrent disease for 1–6 years from the onset of chemotherapy. Conclusions There is no definite answer as to whether older age is a poor prognostic factor in patients with a tumor of the Ewing's sarcoma family. In our experience, patients over 50 poorly tolerated the standard chemotherapy protocol used in the pediatric population.

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Ewing's Sarcoma of the Cervical Spine

Journal of Pediatric Intensive Care ◽

10.1055/s-0037-1606256 ◽

2017 ◽

Vol 07 (02) ◽

pp. 086-089

Author(s):

Meenakshi Bhattacharjee ◽

Winston Huh ◽

Vandana Thapar ◽

Paul Dahm

Keyword(s):

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Pediatric Population ◽

Signs And Symptoms ◽

Neck Mass ◽

Neurological Deficits ◽

Resonance Imaging ◽

Neck Trauma ◽

Motor Weakness ◽

Posterior Neck

AbstractIn this article, we present the case of a 6-year-old female presented to the emergency department with progressive ascending motor weakness leading to cardiac arrest. The recent medical history included neck trauma 1 month prior to admission, 2 weeks of subjective fevers, and 1 day of urinary incontinence. After stabilization, and a review of the recent signs and symptoms, a magnetic resonance imaging of the neck revealed a posterior neck mass from C2 to T2. Neurosurgical removal of the mass was consistent with Ewing's sarcoma. Neck pain is a common presentation in the pediatric population, with the most common cause being traumatic. When coupled with neurological deficits, further studies are warranted to evaluate for organic causes.

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Two rare presentations and treatments of Ewing's sarcoma: A need for a standard treatment approach.

Journal of Clinical Oncology ◽

10.1200/jco.2013.31.15_suppl.e21000 ◽

2013 ◽

Vol 31 (15_suppl) ◽

pp. e21000-e21000

Author(s):

Kanyalakshmi Ayyanar ◽

Nitya Narayan ◽

Karen Moeller ◽

William Gump ◽

Thomas Moriarty ◽

...

Keyword(s):

Local Control ◽

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Residual Disease ◽

Standard Of Care ◽

Diagnostic Techniques ◽

Salvage Chemotherapy ◽

Molecular Diagnostic ◽

Molecular Diagnostic Techniques ◽

Mri Scans

e21000 Background: We describe two Caucasian boys with rare presentations of non-metastatic Ewings sarcoma (ES) with atypical primary sites. One was extradural but FISH negative for 22q11.2. Another was intradural but extramedullary, and FISH positive. Literature search showed few publications which describe such cases, and there is currently no standard of care. Methods: MG, an 11 year old male, presented with a frontal skull mass, with an intracranial epidural extension of about 5 mm. Biopsy confirmed ES. MG was treated with Vincristine, Doxorubicin, and Cyclophosphamide, alternating with Ifosfamide and Etoposide every 2 weeks, for 6 weeks. He underwent tumor excision for local control with good response. Chemotherapy continued, completing 14 cycles total. He is in remission 15 months after completion. AH, a 13 year old male, presented with lower back pain and paraesthesias of feet. MRI revealed an intradural, extra-medullary L1-L3 mass. Intra-operatively, all visible tumor peeled off readily, except for the L2 nerve root. The tumor was FISH positive for EWSR1. He was treated with the same regimen as MG for 6 cycles. Local control was done with radiation. He then continued chemotherapy, completing a total of 14 cycles. MRI scans 3 months later revealed enhancing intradural lesions along the spinal cord (involving C5, T5 and T8) and two lesions along the cerebellum. He was started on cyclophosphamide and topotecan and has completed 8 cycles without disease progression. Results: MG was diagnosed based solely on histo-pathological diagnosis. While MG’s lesion was extra-osseus in nature, more unusual presentations demonstrate the importance of molecular diagnostic techniques. AH was diagnosed based on chromosomal analysis. Progression is presumably from microscopic residual disease in intradural space that invaded CSF. He is presently on salvage chemotherapy. Nine months after relapse, disease is stable, with plans to minimize disease and consolidate. Conclusions: Intradural tumors tend to progress rapidly, maybe more so than extradural tumors. Future suggestions include developing a standard approach that will improve both treatment of Ewing’s sarcoma and prognostic outcomes for rare initial presentations.

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PEDIATRIC EWING'S SARCOMA – AN EXPERIENCE IN A TERTIARY CANCER CARE CENTER IN NORTH EAST INDIA

10.36106/gjra/0800386 ◽

2020 ◽

pp. 6-8

Author(s):

Munlima Hazarika ◽

Satya S Sarangi ◽

Bhargab J Saikia ◽

Partha S Roy ◽

Bibhuti Bhusan Borthakur ◽

...

Keyword(s):

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Care Center ◽

Treatment Protocol ◽

Common Site ◽

Female Ratio ◽

North East India ◽

North East ◽

Male Female Ratio ◽

Incomplete Treatment

Due to limited clinical data in paediatric Ewing’s sarcoma, the aim of this study was to evaluate the demographic characteristics and identifying prognostic factors for survival. We retrospectively reviewed 66 patients with paediatric Ewing’s sarcoma. Median age of presentation was 10 years. Male: Female ratio was 1:1.Femur was the most common site of involvement 13.6% (9/66). The median survival in this study was 52 months. Three and five year survival rate of these patients was 52% and 45% respectively. One out of the 7 patients (14.2%) who did not initiate treatment was alive at the time of analysis whereas 4 out of 8 (50%) patients were alive with incomplete treatment and 35 out of 51 patients (68.6%) were alive who completed treatment and this difference was statistically significant (p<.001).Those patients who completed the treatment protocol had a better survival as compared to those who defaulted or refused treatment.

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Tumor Vessel Development and Expansion in Ewing's Sarcoma: A Review of the Vasculogenesis Process and Clinical Trials with Vascular-Targeting Agents

Sarcoma ◽

10.1155/2011/165837 ◽

2011 ◽

Vol 2011 ◽

pp. 1-7 ◽

Cited By ~ 16

Author(s):

Keri S. Stewart ◽

Eugenie S. Kleinerman

Keyword(s):

Bone Marrow ◽

Clinical Trials ◽

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Pediatric Population ◽

Vascular Supply ◽

Molecular Regulation ◽

Vascular Targeting ◽

Tumor Vessel ◽

Vessel Development

Ewing's sarcoma accounts for a disproportionately high portion of the overall pediatric mortality rate compared to its rare incidence in the pediatric population. Little progress has been made since the introduction of traditional chemotherapies, and understanding the biology of the tumor is critical for developing new therapies. Ewing's sarcomas rely on a functional vascular supply, which is formed by a combination of angiogenesis and vasculogenesis. Recent insights into the molecular regulation of bone marrow (BM) cell participation in vascular development have identified VEGF, SDF-1α, and DLL4 as critical players in the vasculogenesis process. Clinical trials using vascular targeting agents, specifically targeting VEGF or DLL4, are underway.

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