Lethal Leg Pain - Page #4
 

Working Diagnosis:
He was diagnosed with extraosseous Ewing's Sarcoma.

Treatment:
He underwent chemotherapy and radiation. He was not a surgical candidate.

Outcome:
He suffered many treatment and disease related complications, including neutropenic fever and metastases to the lungs, the skull base and surrounding musculature. He underwent radiation of the brain, chest and lumbar region. He passed away from respiratory failure 11 months after initial presentation.

Author's Comments:
Extraosseous bone sarcomas, such as Ewing's Sarcoma, account for 3.6% of soft tissue sarcomas (1). Ewing's Sarcoma most commonly occurs in adolescents and is more common in males than females (2). These tumors may present in a different way than a primary bone tumor, with affected structures determining symptomology. These sarcomas may be at an advanced stage at the time of diagnosis. Constitutional symptoms, such as fevers and night sweats do not generally manifest unless metastases are present. Diagnosis is based on immunohistologic findings. Poor prognosis is associated with extraosseous Ewing's Sarcoma and with metastatic disease at presentation (3). This case is a reminder to maintain a broad differential diagnosis when evaluating the athlete with hip and knee pain.

Editor's Comments:
Night pain, while present in only about 20% of patients presenting with either Ewing sarcoma or osteosarcoma (4), should raise suspicion for one of these diseases or another malignancy if present. Normally plain films will reveal a bony abnormality in the case of Ewing sarcoma, but unfortunately that was not the case in this patient as he did not have bony involvement at presentation.

References:
1. "Cancer Stat Fact Sheets." Surveillance, Epidemiology, and End Results Program. Web: 6 Nov, 2014.
2. Herzog, CE. Overview of sarcomas in adolescent and young adult population. J Pediatr Hematol Oncol 2005. 27: 215-218.
3. Tural, Deniz, Mandel, Nil M, et al. Extraosseous ewing's sarcoma family of tumors in adults: prognostic factors and clinical outcomes. Japanese Journal of Clinical Oncology 2012. 42(5): 420-426.
4. Widhe B, Widhe T. Initial Symptoms and Clinical Features in Osteosarcoma and Ewing Sarcoma. J Bone Joint Surg Am, 2000 May; 82(5): 667-674.

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