Ewing Sarcoma in a Young Adult: Survival following Disease Recurrence




Basha, Riyaz
Wise, Matthew
Cope-Yokoyama, Sandy
Bowman, Paul


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Background: Ewing sarcoma is a rare, malignant cancer of bone and soft tissue that primarily affects adolescents and young adults. It is generally considered a treatable cancer. Nevertheless, when patients defy traditional survival indicators, namely age at diagnosis, relapse occurrence, and disease-free interval, their management, especially prognostically, is all the more challenging. Case Presentation: A 28-year-old Caucasian male presented to his physician with complaints of intermittent, severe chest pain and fever over 14 months. A right intrathoracic soft tissue mass was found on computed tomography (CT scan), and biopsy identified a small round blue cell tumor. The patient sought a 2nd opinion at Cook Children’s Medical Center, where bone scan and pathology confirmed an invasive Ewing sarcoma of the 5th rib. Ninety-five percent of cases with this diagnosis are made in patients who are between the ages of 4 and 25 years old. One week later, he began intensive chemotherapy at 2 week intervals (interval compression protocol) of 14 cycles, alternating Vincristine, Doxorubicin, and Cyclophosphamide with Ifosfamide and Etoposide. Ribs 4-6 were resected after completion of the 6th cycle, and tumor showed greater than 90% necrosis, indicating a dramatic response to chemotherapy. Following surgery, the patient completed 8 more cycles with excellent response and minimal complications. However, the patient presented again with recurrent chest pain 24 months later. Magnetic Resonance Imaging (MRI) and tissue biopsy confirmed tumor relapse along the 5th rib. Immediately, he began 12 cycles of Irinotecan & Temozolomide on an interval compression schedule. The tumor was successfully resected after the 4th cycle, and radiation to the site was added during the 9th and 10th cycles. Since his recurrence, the patient has shown no signs of relapse or metastasis during a follow-up period of 4 years and 10 months. This favorable outcome contrasts with the less than 20% survival rate anticipated in local recurrences between 1-2 years after complete remission. Conclusions: This case is unique in that the patient presented outside the expected age range, was diagnosed more than a year after the onset of symptoms, and relapsed within two years of complete remission. Yet, he survives several years later, free of disease and with minimal complications. This case challenges the current understanding of prognostic factors in Ewing sarcoma while emphasizing their influence in the proper care and management of patients.