Ewing’s Sarcoma of the Vertebral Body in an Adolescent: A Rare Case Report and Literature Review (original) (raw)
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Primary Ewing's Sarcoma of the Spine: Four Case Report
KYAMC Journal, 2019
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 Jou...
Primary Ewing Sarcoma of Lumbar Spine With Massive Intraspinal Extension
Pediatric Neurology, 2008
Primary vertebral Ewing sarcoma-primitive neuroectodermal tumor is uncommon. Although epidural extension has been seen in such tumors, cases with massive intraspinal involvement are decidedly rare. Here we present the case of a 4-year-old girl with back pain and difficulty walking. Magnetic resonance imaging showed a mass filling the spinal canal from T 11 to the L 3 /L 4 levels. Vertebral involvement with extension into the paraspinal soft tissue through neural foramina was seen. Histologically, a small-blue-cell tumor with strong membranous CD99 reactivity was noted. Molecular analysis revealed translocation t(11;22)(q24;q12), thus confirming the diagnosis of Ewing sarcoma-primitive neuroectodermal tumor. Our case emphasizes that vertebral Ewing sarcoma-primitive neuroectodermal tumor may present with massive intraspinal extension and should be included in the differential diagnosis of intraspinal lesions.
Primary Ewing sarcoma of the spine: Report of two cases
Annals of Saudi medicine, 1993
Ewing sarcoma is a primary bone tumor which was originally described by Lucke in 1866 but was defined in such detail by Sir James Ewing in 1921 that it is now associated with his name [1]. This tumor involves the spine primarily in 3.5% to 14.9% r~~ases [2,3]. The review of 22 cases of Ewing Sarcoma of the spine by Pilepich et al in 1981 [4] remains the largest study of this pathology in such location in the literature and apart from a few smaller series [3,5], most of the documentation of Ewing sarcoma of the spine has been by isolated case reports [6-8]. Considering the worldwide rarity of primary spinal Ewing sarcoma, we were surprised to encounter two cases among the 30 cases of spinal tumors treated in our unit over the last seven years [9]. There are a number of articles reflecting on the experience with Ewing sarcoma in Saudi Arabia [10-12]. However, to our knowledge, in none of these reports was the spine the primary site of the tumor. We feel justified, therefore, in presenting our t•..') cases to highlight awareness of clinicians to the v~..;asionalinvolvement of the spine as a primary site of Ewing sarcoma, which should be considered in the differential diagnosis of spinal tumors causing cord compression. Case Histories The clinical characteristics of the two cases appear in the Table (Figures 1-3). The chest x-ray, abdominal ultrasound, skeletal survey and isotope bone scans for both cases were normal.
A Case of Ewing's Sarcoma Involving Cervical Spine
Ewing's sarcoma is a highly malignant tumour involving the long bones of extremities together with soft tissues. Our case reports a very unusual case of Ewing's sarcoma of spine. In this report, a 11 years old boy who presented with features of cold compression. After reaching a diagnosis of a space occupying lesion in cervical region, we excised the maps surgically and biopsy was sent which confirmed our diagnosis of swings sarcoma. Surgery was followed by dramatic improvement in motor signs of patient and he was referred to NIMRA for further treatment with radiotherapy and chemotherapy.
Rare entities are difficult to predict. They are considered last rightly, to expedite treatment and alleviate symptoms quickly. Rare presentations of rare diseases form a particularly difficult section of diagnoses that are not only impossible to predict but difficult to recognize, diagnose, and treat. Often the dilemma is to, investigate thoroughly saving time but financially burdening the patient and hospital, or, to investigate in gradual increments taking more time and effort, especially in rare cases where prolonged hospitalization and suffering occurs before the diagnosis is reached. This approach, however, wastes critically important time, which, especially in neurological compression, may often lead to irreversible deficits. This dilemma is admirably demonstrated in this case report of spinal Ewing's sarcoma. A young female presented to us with recurrent high cervical epidural collections presenting as compressive myelopathy. She underwent repeated decompressions, and the collection was misdiagnosed as tuberculosis, which was treated without empirical evidence, leading to significant irreversible disability. Finally, when she came to us, the histopathological assessment was done to reveal the diagnosis. Ewing's sarcomas, and indeed the whole gamut of small-round-cell malignancies, are great imitators. They are known to exist in the skull base mimicking schwannomas, chordomas, germinomas, pituitary adenomas, and even epidermoids and occasionally extend to the vertebral bodies and the cranio-vertebral Junction (CVJ) leading to instability and neurological compression. Here, they mimic vertebral tumors, discitis, infective abscesses, and even myeloma. Predictably, such an entity is diagnosed last, and diagnosed late, leading to bad consequences for the patient. Such was the fate of our patient. The report emphasizes the diagnostic dilemma and presents the need to use protocols for diagnosis and treatment, even in rare cases, to effect the best possible outcomes for patients. The use of a thorough diagnostic and management algorhythm prevents deeper and sinister disease processes from being missed.
Ewing's Sarcoma in the Spinal Nerve Root: A Case Report and Review of the Literature
The Tohoku Journal of Experimental Medicine, 2006
Ewing's sarcoma (ES) is a highly malignant tumor composed of uniform small round cells. Recently, a single biologic entity, Ewing's sarcoma family of tumors (ESFT) has been accepted. The entity includes ES, extraskeletal Ewing's sarcoma (EES) and primitive neuroectodermal tumor (PNET). ESFT cells have immunoreactivity for CD99, an antigen determined by the MIC2 gene. Most ESFT has the (11;22) (q24;q12) translocation. The translocation results in the fusion of the EWS gene with the transcription factor gene FLI1 which has been considered a hallmark of ESFT. We present an extremely unusual case with ESFT in a spinal nerve root mimicking a neurogenic dumbbell tumor. A male aged 20 years noticed pain in his right buttock. Magnetic resonance imaging (MRI) revealed a mass in the right L5/S intervertebral foramen and the lesions in the sacrum. Surgery was performed with a presumptive diagnosis of a nerve sheath tumor. At surgery, the tumor was located in the right L5 nerve root sleeve. The sacral lesions were observed closely. At one month after surgery, radiologically multiple lesions were detected in the pelvic bones. Microscopically the lesions from the root and ilium were composed of small round cells immunoreactive for CD99. Reverse transcription-polymerase chain reaction detected transcripts resulting from the fusion of the EWS gene with FLI1 genes in the iliac lesion. Immunoreactivity for CD99 and detection of the EWS-FLI1 hybrid transcripts are important for the correct diagnosis of ESFT arising in an unusual location. Ewing's sarcoma; Ewing's sarcoma family of tumors; extraskeletal Ewing's sarcoma (EES); primitive neuroectodermal tumor (PNET); nerve root
Paraplegia: an unusual presentation of Ewing's sarcoma
Spinal Cord, 2002
Study design: Single-subject case. Objectives: To describe the atypical presentation of a rare tumor and diculties in diagnosis. Setting: Spinal cord unit of a rehabilitation and care center in Ankara, Turkey. Methods: A 22-year-old male patient with paraplegia was admitted to our center for rehabilitation. He underwent various diagnostic procedures to explain his clinical situation. Results: Abdominal ultrasound and computed tomography revealed a mass and vertebral destruction. The pathology report con®rmed the diagnosis of Ewing's sarcoma. Conclusion: Spinal cord compression is an important complication of primary or metastatic malignant tumors. Although it is a late complication in most cases, some tumors including Ewing's sarcoma may present with paraplegia. Trauma may be found in the patient's history. An atypical clinical course in a musculoskeletal or neurological condition should alert us to a possible underlying malignant disease.
Primary Intramedullary Ewing’s Sarcoma: A Case Report and Review of the Literature
Case Reports in Clinical Medicine, 2015
Ewing's sarcoma, a group of primary neuroectodermal tumor (PNET), is an uncommon rare highly malignant tumor mostly affecting the bones of male patients, and accounts for approximately 10% of the primary malignant bone tumors, and occupies the second place after osteosarcoma in children's less than 20 years. Primary intramedullary Ewing's sarcoma is an extremely rare condition. Here, we present a case of a 16-year-old girl with progressive weakness of lower extremities, inability to walk, progressive low back pain, bilateral leg pain, paresthesia and urinary retention. Spinal MRI showed intramedullary abnormal signal focus from T11 to L3 vertebrae with moderate heterogeneous enhancement on T1 weighted sequences. After surgery, histological examination found a lot of small round-cell tumors with a high nuclear-cytoplasmic ratio, frequent mitoses and apoptotic nuclei suggesting a diagnosis of Ewing's sarcoma and confirmed by CD99 reactivity. She had undergone adjuvant chemotherapy and irradiation and was free of symptoms since 02 years. Within this single pure case of intramedullary Ewing's sarcoma, perhaps the 1st in the English literature, the authors described this extremely rare uncommon localization with a brief review of the literature.