A Rare Presentation Of Ewing's Sarcoma Involving Spine With Acute Paraplegia

Ewing's sarcoma is a primary bone malignancy with the highest incidence in the first decade of life. Although it mostly affects the diaphyseal as well as metaphysical region of growing long bones but involvement of spine is not very uncommon especially the sacrum. Nonsacral spinal Ewing's sarcoma is rarer and often mimics a benign condition before spreading extensively. They present with neurologic deficits due to spinal cord compression, but acute onset paraplegia is very rare.A high index of clinical suspicion can clinch the diagnosis early in the course of the disease. A prompt intervention is required to keep neurological damage to a minimum, and a correct combination of surgery, chemotherapy, and radiotherapy is required for better long-term patient outcome. We report a 15-years old male who presented with acute paraplegia and had an excellent postoperative outcome after posterior decompression of a D 11 Ewing's sarcoma.TAJ 2013; 26: 92-97

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A rare case of acute paraplegia: primary Ewing’s Sarcoma of dorsal spine

International Surgery Journal ◽

10.18203/2349-2902.isj20181460 ◽

2018 ◽

Vol 5 (5) ◽

pp. 1927

Author(s):

Yeshwant Kumar N. N. T. ◽

V. T. T. Rajan

Keyword(s):

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Postoperative Outcome ◽

Cord Compression ◽

Acute Onset ◽

Radical Excision ◽

Benign Condition ◽

Acute Paraplegia ◽

Primary Bone

Ewing's sarcoma is a primary bone malignancy with the highest incidence in the second decade of life. Although it mostly affects the metaphyseal region of long growing bones, involvement of spine is not very uncommon especially the sacrum. Non-sacral spinal Ewing's sarcoma is rarer and often mimics a benign condition before spreading extensively. They present with neurologic deficits due to spinal cord compression, but acute onset paraplegia has not been previously reported. A high index of clinical suspicion can clinch the diagnosis early in the course of the disease. A prompt intervention is required to keep neurological damage to a minimum, and a correct combination of surgery, chemotherapy, and radiotherapy is required for better long-term patient outcome. We report a 11-year-old female who presented with acute paraplegia and had an excellent postoperative outcome after radical excision of a C7-D2 Ewing's sarcoma.

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Primary Ewing's Sarcoma of the Spine: Four Case Report

KYAMC Journal ◽

10.3329/kyamcj.v10i3.44423 ◽

2019 ◽

Vol 10 (3) ◽

pp. 164-167

Author(s):

Md Mofazzal Sharif ◽

Khaleda Parvin ◽

Umme Iffat Siddiqua ◽

A.Q. Mehedi Hassan ◽

Jabed Hossain ◽

...

Keyword(s):

Soft Tissues ◽

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Correct Diagnosis ◽

Surgical Excision ◽

Cord Compression ◽

Acute Paraplegia ◽

Low Incidence ◽

Specific Symptoms ◽

Common Malignancy

Ewing's sarcoma is a common malignancy of the bone and soft tissues in pediatric patients. It mostly affects the long bones and pelvis, and less commonly the flat bones and vertebrae. Primary Ewing's sarcoma affecting the spine is very rare. The patient has non-specific symptoms for a prolonged period of time before the correct diagnosis is given. Patients can present with acute paraplegia due to spinal cord compression, which needs prompt surgical intervention. Early diagnosis and treatment are important for neurological recovery. The definitive management includes three main modalities: surgery, radiotherapy and combination chemotherapy. Adequate surgical excision may not be feasible because of anatomical limitations and local control is mainly achieved by radiotherapy. Because of the low incidence of these tumors, a multitude of therapeutic strategies have been employed with varying success. Currently there are no clinical guidelines outlining optimal management KYAMC Journal Vol. 10, No.-3, October 2019, Page 164-167

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A Unique Case of Primary Ewing’s Sarcoma of the Cervical Spine in a 53-Year-Old Male: A Case Report and Review of the Literature

Case Reports in Medicine ◽

10.1155/2015/402313 ◽

2015 ◽

Vol 2015 ◽

pp. 1-6 ◽

Cited By ~ 7

Author(s):

Marshall T. Holland ◽

Oliver E. Flouty ◽

Liesl N. Close ◽

Chandan G. Reddy ◽

Matthew A. Howard

Keyword(s):

Cervical Spine ◽

Spinal Canal ◽

Ewing’S Sarcoma ◽

Treatment Guidelines ◽

Ewing's Sarcoma ◽

Histopathological Examination ◽

Acute Onset ◽

Rare Presentation ◽

Low Incidence ◽

Histopathological Studies

Extraskeletal Ewing’s sarcoma (EES) is a rare presentation, representing only 15% of all primary Ewing’s sarcoma cases. Even more uncommon is EES presenting as a primary focus in the spinal canal. These rapidly growing tumors often present with focal neurological symptoms of myelopathy or radiculopathy. There are no classic characteristic imaging findings and thus the physician must keep a high index of clinical suspicion. Diagnosis can only be definitively made by histopathological studies. In this report, we discuss a primary cervical spine EES in a 53-year-old man who presented with a two-month history of left upper extremity pain and acute onset of weakness. Imaging revealed a cervical spinal canal mass. After undergoing cervical decompression, histopathological examination confirmed a diagnosis of Ewing’s sarcoma. A literature search revealed fewer than 25 reported cases of primary cervical spine EES published in the past 15 years and only one report demonstrating this pathology in a patient older than 30 years of ageage=38. Given the low incidence of this pathology presenting in this age group and the lack of treatment guidelines, each patient’s plan should be considered on a case-by-case basis until further studies are performed to determine optimal evidence based treatment.

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Primary Bone Tumours of the Pelvis in Childhood—Ewing's Sarcoma of the Ilium, Pubis and Ischium. (Report of 30 cases) (Part I)

Australasian Radiology ◽

10.1111/j.1440-1673.1989.tb03312.x ◽

1989 ◽

Vol 33 (4) ◽

pp. 354-360 ◽

Cited By ~ 4

Author(s):

K. KOZLOWSKI ◽

J. CAMPBELL ◽

G. BELUFFI ◽

J.C. HOEFFEL ◽

L. MORRIS ◽

...

Keyword(s):

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Bone Tumours ◽

Primary Bone ◽

Primary Bone Tumours

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A long-term review of the treatment of patients with Ewing's sarcoma in one institution

European Journal of Surgical Oncology ◽

10.1053/ejso.2001.1164 ◽

2001 ◽

Vol 27 (6) ◽

pp. 569-573 ◽

Cited By ~ 21

Author(s):

M. Sluga ◽

R. Windhager ◽

S. Lang ◽

H. Heinzl ◽

P. Krepler ◽

...

Keyword(s):

Ewing’S Sarcoma ◽

Ewing's Sarcoma

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Long-term follow-up for patients with Ewing's sarcoma of bone treated with adjuvant and neoadjuvant chemotherapy

Oncology Reports ◽

10.3892/or.4.5.977 ◽

1997 ◽

Author(s):

G Bacci ◽

S Ferrari ◽

E Barbieri ◽

A Longhi ◽

C Forni ◽

...

Keyword(s):

Neoadjuvant Chemotherapy ◽

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Long Term Follow Up

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Multimodal therapy for the management of primary, nonmetastatic Ewing's sarcoma of bone: a long-term follow-up of the First Intergroup study.

Journal of Clinical Oncology ◽

10.1200/jco.1990.8.10.1664 ◽

1990 ◽

Vol 8 (10) ◽

pp. 1664-1674 ◽

Cited By ~ 376

Author(s):

M E Nesbit ◽

E A Gehan ◽

E O Burgert ◽

T J Vietti ◽

A Cangir ◽

...

Keyword(s):

Overall Survival ◽

Local Recurrence ◽

Multimodal Therapy ◽

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Patient Characteristics ◽

Group I ◽

Long Term Follow Up

A total of 342 previously untreated eligible children were entered into the first Intergroup Ewing's Sarcoma Study (IESS) between May 1973 and November 1978. In group I institutions, patients were randomized between treatment 1 (radiotherapy to primary lesion plus cyclophosphamide, vincristine, dactinomycin, and Adriamycin [doxorubicin; Adria Laboratories, Columbus, OH] [VAC plus ADR]) or treatment 2 (same as treatment 1 without ADR), and group II institutions randomized patients between treatment 2 or treatment 3 (same as treatment 2 plus bilateral pulmonary radiotherapy [VAC plus BPR]). The percentages of patients relapse-free and surviving (RFS) at 5 years for treatments 1, 2, and 3 were 60%, 24%, and 44%, respectively. There was strong statistical evidence of a significant advantage in RFS for treatment 1 (VAC plus ADR) versus 2 (VAC alone) (P less than .001) and 3 (P less than .05) and also of treatment 3 versus 2 (P less than .001). Similar significant results were observed with respect to overall survival. Patients with disease at pelvic sites have significantly poorer survival at 5 years than those with disease at nonpelvic sites (34% v 57%; P less than .001). Among pelvic cases, there was no evidence of differing survival by treatment (P = .81), but among nonpelvic cases, there was strong evidence of differing survival by treatment (P less than .001). The overall percentage of patients developing metastatic disease was 44%; the percentages by treatments 1, 2, and 3 were 30%, 72%, and 42%, respectively. The overall incidence of local recurrence was 15%, and there was no evidence that local recurrence rate differed by treatment. Patient characteristics related to prognosis, both with respect to RFS and overall survival experience, were primary site (nonpelvic patients were most favorable) and patient age (younger patients were more favorable).

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