Primitive neuroectodermal tumors of the spine: a comprehensive review with illustrative clinical cases

Ellis, Jason A.; Rothrock, Robert J.; Moise, Gaetan; McCormick, Paul C.; Tanji, Kurenai; Canoll, Peter; Kaiser, Michael G.

doi:10.3171/2010.10.focus10217

Cited by 51 publications

(96 citation statements)

References 85 publications

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“…The peak age for EWS is 15 to 17 years, being rarer in adults [1,7,8,9]. Primary Ewing's sarcoma of spine has male preponderance with posterior elements of the spine being commonly involved and are mostly extradural-extramedullary.…”

Section: Discussionmentioning

confidence: 99%

“…The occurrence in adult age is quite rare [1,7]. The most common primary sites of involvement are the pelvis, femur, tibia, and fibula.…”

Section: Introductionmentioning

confidence: 99%

“…The translocation t(11; 22) (q24; q12) is identified in more than 90% of cases of EWS which help differentiate it from other small, round blue cell tumors [1,2].…”

Section: Introductionmentioning

confidence: 99%

“…The Ewing family of tumors comprises Ewing's sarcoma (EWS), extraskeletal EWS, primitive neuroectodermal tumor (PNET) of bone and soft tissue, and chest wall tumor (Askin tumor) [1,2,3,4]. The translocation t(11; 22) (q24; q12) is identified in more than 90% of cases of EWS which help differentiate it from other small, round blue cell tumors [1,2].…”

Section: Introductionmentioning

confidence: 99%

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Atypical Presentation of Primary Non-metastatic Ewings Sarcoma of the Lumbosacral Spine-“Cauda Equina Syndrome” Clinically and a “Tuberculosis mimicking challenge” on MRI-A Case Report

Kukreja¹,

Telang²,

Anchan³

2017

AJMCR

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Introduction: The Ewing family of tumors comprises Ewing's sarcoma (EWS), extraskeletal EWS, primitive neuroectodermal tumor (PNET) of bone and soft tissue, and chest wall tumor (Askin tumor). The translocation t(11; 22) (q24; q12) is identified in more than 90% of cases.EWS is the second most common primary bone malignancy in childhood. In contrast to long bone involvement, delays in spinal EWS diagnosis may occur because symptoms may not be present until neurological deficits occur. To date, although there have been reported cases of EWS in the lumbosacral region, the reported cases are very less and the presentations might drastically differ. Case Presentation: A 14 year old boy comes with a 12 weeks h/o urinary incontinence and constipation. Over the next 4 months, the patient had new-onset intermittent abdominal pain with worsening urinary symptoms.MRI confirmed a diagnosis of Koch's spine. However, the histopathology reported it as an "Ewings sarcoma/Primitive Neuroectodermal Tumor". Discussion: Ewing's tumor of sacrum is rare, but should be suspected in low backache in children. Cauda equina syndrome can be a valid presentation for EWS spine. MRI can identify cases early and enables early treatment though it is not specific. Histopathological diagnosis is a must before any definitive management.EWS spine can mimick Tuberculosis both clinico-radiologically, only to be confirmed with a tissue biopsy. Conclusion: Ewing's sarcoma of the lumbosacral spine can have an atypical presentation and there should be a high degree of suspicion to diagnose it early.

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Section: Discussionmentioning

confidence: 99%

“…The occurrence in adult age is quite rare [1,7]. The most common primary sites of involvement are the pelvis, femur, tibia, and fibula.…”

Section: Introductionmentioning

confidence: 99%

“…The translocation t(11; 22) (q24; q12) is identified in more than 90% of cases of EWS which help differentiate it from other small, round blue cell tumors [1,2].…”

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Atypical Presentation of Primary Non-metastatic Ewings Sarcoma of the Lumbosacral Spine-“Cauda Equina Syndrome” Clinically and a “Tuberculosis mimicking challenge” on MRI-A Case Report

Kukreja¹,

Telang²,

Anchan³

2017

AJMCR

View full text Add to dashboard Cite

show abstract

“…The Ewing's family of tumours comprise of Ewing's sarcoma, extraskeletal Ewing's sarcoma, primitive neuroectodermal tumour (PNET) of bone and soft tissue and chest wall tumour (A skin's tumour).The translocation t (11;22) (q24;q12) is identified in more than 90% of cases of Ewing's sarcoma [5,6]. Spinal involvement most commonly results from metastasis in advanced stages of the disease, while Ewing's sarcoma originating from the spine is rare and extremely rare if the sacrum is excluded [7].…”

Section: Discussionmentioning

confidence: 99%

Unusual Presentation of a Primary Ewing’s Sarcoma of the Spine with Paraplegia: A Case Report

Kannan¹

2015

JCDR

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An 18-year-old male was admitted with severe back pain and inability to move both his lower limbs for the past two months. Back pain was insidious in onset, progressive and pain was present during rest. Two months back, patient noticed weakness of both lower limbs while walking which progressed to complete inability to move both lower limbs for the past one month. Patient gave history of intermittent low grade fever and weight loss in the past two months. He also gave history of bowel and bladder incontinence for the past one month. There was no contact history for tuberculosis. He had tenderness in the lower back and neurological examination revealed Grade 0 power in both lower limbs with hypotonia. Sensory loss started from mid thigh to involve the legs, ankles and feet of both lower limbs. The knee jerk and ankle jerk were absent and there was no response for plantar reflex. Patient had stage three pressures sore over his sacrum. His hemoglobin was 10.5 gm% with increase in the total WBC count and erythrocyte sedimentation rate. Other laboratory investigations were normal.His radiographs showed no significant abnormality, MRI [ Fig-4] showed hyperintense T2 signal from L4 body with soft tissue enhancement involving both the psoas muscles, fluid collection in the paraspinal muscles and an extension into the spinal canal from L2 to L5 vertebrae causing a complete block in the myelogram. The disc spaces were normal and there were no skip lesions on the MRI. The radiologist had given a differential diagnosis with infective spondylitis being the first diagnosis and malignant pathology being the subsequent diagnosis.Patient was taken up for posterior spinal surgery, through a posterior midline incision L2 to L5 vertebra were exposed and there was frank pus in the paraspinal region which was collected for culture and sensitivity. Decompressive laminectomy was performed at L3 and L4 vertebra to enter the spinal canal. There was a layer of granulation tissue covering the dura as in tuberculosis pathology, which was carefully separated from the dura and sent for histopathological analysis. Transpedicular bone sample obtained from L4 vertebra and was sent for histopathological analysis. The levels were stabilized Postoperatively the patient had good pain relief and was made to sit up with a brace on the second postop day. Neurology was reassesed and the motor power in both lower limbs were zero and sensory loss remained the same too. However, the patient had full recovery of bowel and bladder function. HistopatHology Microscopic DescriptionSections show fibrocollagenous stroma and skeletal muscle fibres infiltrated by a malignant neoplasm arranged in diffuse sheaths, nests and pseudo acinar pattern. Ewing's sarcoma is a primary malignancy of the bone affecting individuals in the second decade of life. Primary sarcomas of the spine are rare and the occurrence of Primary Ewing's sarcoma in the spine is very rare. Ewing's sarcoma occurring in the spine is divided into two types, Ewing's sarcoma of sacral spine which are v...

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Modern surgical techniques for management of soft tissue sarcomas involving the spine: Outcomes and complications

Mattei¹,

Teles

Mendel

2014

Journal of Surgical Oncology

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Several types of soft tissue sarcomas may locally extend to the spine. The best therapeutic strategy for such lesions strongly depends on the histological diagnosis. In this article the authors provide an up-to-date review of current guidelines regarding the management of soft tissue sarcomas involving the spine. Special attention is given to outcomes and complications of modern surgical series in order to highlight current challenges in the management of such lesions.

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Primitive neuroectodermal tumors of the spine: a comprehensive review with illustrative clinical cases

Cited by 51 publications

References 85 publications

Atypical Presentation of Primary Non-metastatic Ewings Sarcoma of the Lumbosacral Spine-“Cauda Equina Syndrome” Clinically and a “Tuberculosis mimicking challenge” on MRI-A Case Report

Atypical Presentation of Primary Non-metastatic Ewings Sarcoma of the Lumbosacral Spine-“Cauda Equina Syndrome” Clinically and a “Tuberculosis mimicking challenge” on MRI-A Case Report

Unusual Presentation of a Primary Ewing’s Sarcoma of the Spine with Paraplegia: A Case Report

Modern surgical techniques for management of soft tissue sarcomas involving the spine: Outcomes and complications

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