Spinal intradural metastasis from scapular Ewing sarcoma

Abstract

BackgroundEwing 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 presentationWe 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.ConclusionIntradural 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.

Ewing's sarcoma in scapula, epidemiology, clinical manifestation, diagnosis and treatment: A literature review

“…Swelling was associated with pain in 26.67% of the cases. In a few cases, pre-metastases [ 21 ] and lower limb weakness [ 22 ] were the main presentations. As swelling was the main and most common presentation, shoulder movement restriction was expected to be a presentation in 33.33% of the cases ( Table 2 ).…”

“…In general, no neurovascular deficits were reported. One case unexpectedly developed neurological retrogradation that involved the upper limb 3 days after the onset of symptoms due to severe cord compression caused by intradural extramedullary mass with an extra-spinal component [ 22 ]. Tumor size was determined through radiographic investigations, such as magnetic resonance imaging (MRI) and computed tomography (CT).…”

“…Local invasion was found in 63.64% of the cases, and premetastases were observed in 35.71% of the cases. Metastases were observed in the lungs (4/15) [ 4 , 20 , 21 , 26 ], lymph nodes (1/15) [ 4 ], bone marrow (1/15) [ 27 ], skeleton (1/15) [ 28 ], and spinal intradural (1/15) [ 22 ].…”

Subdural spinal metastases detected on CT myelography: A case report and brief review

“…Subdural metastases are most often discovered incidentally, because they usually occur only in patients with advanced metastatic disease. However, mass effect from the lesions can cause myelopathic symptoms, including pain, bilateral weakness, and sensory deficits [6] . Bladder dysfunction has also been noted in several cases of intradural metastases, although several of these were intramedullary [7] .…”

Ewing’s Sarcoma: imaging findings of a patient with primary tumor in the femur and mandibular metastasis

“…Other symptoms include fever, anemia and leukocytosis [8]. Radiographic findings of ES show an osteolytic lesion that is not a pathognomonic feature [9].…”