Epidural metastasis of Ewing's sarcoma: a rare localisation (original) (raw)
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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.
Oncology Reports, 2011
The occurrence of primary extraskeletal Ewing's sarcoma (EES) of the spinal epidural space has been rarely reported in the literature. The clinical, radiologic and pathologic features of a case of EES occurring in the thoracic epidural space are presented. A 37-year-old woman presented with a one-year history of back pain. Magnetic resonance imaging demonstrated an epidural mass at the T8-9 level. Laminectomy and partial resection of the tumor were performed. The differential diagnosis of a spinal epidural mass is broad. Histopathological and molecular cytogenetic examinations confirmed an EES arising from the thoracic epidural space. Despite receiving both chemotherapy and radiotherapy, the patient died of respiratory insufficiency due to medulla oblongata metastasis 22 months after the initial consultation. Awareness of this entity will allow this rare diagnosis to be considered and facilitate appropriate management. A review of the literature on spinal epidural EES is also presented.
Spinal intradural metastasis from scapular Ewing sarcoma
BMC Research Notes, 2015
Background: Ewing sarcoma is a primary bone neoplasm, which is a high grade aggressive small round blue cell tumour, and is currently recognized as a part of the Ewing family of tumours. It is the most lethal bone tumor, and is a rare malignant bone tumor accounting for 10% of all primary bone tumors, and 6% of malignant bone tumors. It has an average annual incidence of 3 per 1 million, found almost exclusively in Caucasians. It commonly occurs in long bones and pelvis but rarely involves the scapula. 85% of cases have metastasis within 2 years of diagnosis, rarely involving the meninges. Case presentation: We report a case of a 25 year old Sinhalese Sri Lankan female, presenting with a 1 day history of bilateral lower limb weakness and urinary incontinence. She had a sensory level with flaccid paralysis of lower limbs and a painless bony lump in the left dorsal scapula. Investigations showed scapular primary Ewing sarcoma giving rise to spinal intradural metastasis. For the best of our knowledge this is the first reported case of a scapular Ewing sarcoma with spinal intradural metastasis presenting with lower limb paralysis. Conclusion: Intradural spinal metastasis of Ewing sarcoma presenting with lower limb weakness, without a history of pain, though rarely, can be the first presentation, and can rapidly progress to brainstem involvement and death.
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 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.
Nonmetastatic Ewing’s Sarcoma of the Lumbar Spine in an Adult Patient
Case Reports in Oncological Medicine, 2012
Although the spine is frequently involved in metastatic Ewing's sarcoma, primary involvement of the spine, beside sacrum, is much less frequent, especially in adult patients. Because of the low incidence of these tumors, there are currently no clinical guidelines outlining their management and a multitude of therapeutic strategies have been employed with varying success. The definitive management of Ewing's sarcoma of the spine, as in other locations, could include the combination of three main modalities: aggressive surgery, radiotherapy, and combined chemotherapy. Whenever possible, en bloc spondylectomy or extralesional resection is preferable, providing a better oncological result with a longer survival and a better preservation of the spine biomechanics. This is the lesson we learned about the case, we present here, of nonmetastatic lumbar localization by Ewing’s sarcoma in as adult patient.
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.
Surgical Neurology International, 2012
Background: Primary spinal primitive neuroectodermal tumors (PNET) and/ or spinal extraskeletal Ewing's sarcoma family tumors (ESET) are rare lesions appearing in the spinal extradural space. One hundred forty-one primary spinal PNETs, including 29 intramedullary lesions, have been reported in the literature. Encountering a case of primary epidural EES/peripheral PNET (pPNET) in sacral level, which is the fifth one occurring at this level in the literature, we have tried to conduct a meta-analysis of the reported cases. Case Description: A 44-year-old lady with epidural EES/pPNET is reported here. She was once operated for L5/S1 herniated disc, which did not ameliorate her symptoms. The clinical, imaging, surgical, and histopathologic characteristics of our case are presented and wide search of the literature is also done. All the reports were level 3 or less evidences and most of the series had missing parts. 106 cases of primary intraspinal (extradural/extramedullary-intradural) EES/pPNET and 29 cases of primary intramedullary PNET (CNS-PNET) have been reported in the literature. The most common clinical presentation in both entities was muscle weakness proportionate to the tumor location. Distant metastasis occurred in 38 of 99 (38%) cases of primary intraspinal EES/pPNET, while the rate of metastasis was 48% in patients with PNETs occurring in the intramedullary region (P > 0.05). One-year survival rate of the patients who underwent chemo-radiation after total or subtotal resection was better than those who did not receive chemotherapy or radiotherapy, or did not have total or subtotal resection. However, this difference was not repeated in 2-year survival rate in any of the tumor groups. Conclusion: It seems that total or subtotal removal of the tumor and adjuvant chemo-and radiation therapy can improve the outcome in these patients.
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...