Spinal ewing's Sarcoma presenting as an epidural collection: A rare presentation of a rare entity

Abstract

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.

Apparent Provisional Diagnosis Validated by Histopathology: 2 Case Report that mimics TB Spine Conflict of interest: There is no Conflict of interest relevant to this paper to disclose

“…In tubercular spondylodiscitis, there is the presence of a large adjacent soft tissue component, rim-enhancing abscesses, and bone fragments which usually compress neural elements 10 . These clinic -radiological features are not typical for tubercular spondylodiscitis, rather in various reported articles, these features are consistent with other lesions mimicking Pott's disease [11][12][13][14][15] .…”

“…Rare entities are not only difficult to predict but difficult to recognize, diagnose, and treat. Often the dilemma is to, investigate thoroughly saving time but financially burdening the patientand hospital, or, to investigate in gradual increments taking more time and effort while neurological deficit continued which causes irreversible damage to neural elements [13] . Sharma et al [27] reported that preservation of the intervertebral discarchitecture and lack of destruction of thevertebra are the imaging points in favor of aparaspinal Ewing's sarcoma as compared to aparaspinal tubercular abscess.…”

Primary Ewing Sarcoma of the Cervical Spine: A Case Report and Literature Review

“…Ewing sarcoma is a high-grade small round cell tumor of neuroectodermal origin and it shares the presence of a common chromosomal translocation with other tumors like peripheral primitive neuroectodermal tumor (pPNET), atypical Ewing sarcoma, and Askin tumor [ 13 ]. Most cases of Ewing sarcoma occur in the skeleton (the long bones, pelvis, or ribs) and rarely in extra-skeletal regions such as the paravertebral or epidural space, whereas the primary IEES is extremely rare [ 2 ].…”

Ewing’s Sarcoma of the Craniovertebral Junction - A Rare Cause of Reversible Quadriplegia. A Case Report with a 10-year Review of Literature

“…The final diagnosis requires immunohistochemical analysis and cytogenetic studies to identify (11;22) (q24; q12) translocation. EWS / ETS fusions as the presumed initiating oncogenic event required for proliferation and tumorigenesis [7][8][9][10][11].…”