The longest recurrence-free survival in Ewing's sarcoma patient

What is it about?

The prognosis for patients diagnosed with Ewing's sarcoma (EwS) substantially improved since the integration of chemotherapy (CHT) into treatment protocols in the early 1970s. In the preceding series, the 5-year overall survival rate was documented to be approximately 10%, while in the recent ones it ranges between 75% and 80%. Consequently, the reports of very long-term survivors (≥ 30 years post-treatment) are scarce, particularly in cases where secondary malignancies and pathological fractures are present. Therefore, we present the case of the longest (to our knowledge) survival in EwS reported to date. An 18-year-old female presented with a 10 × 7 × 7 cm tumor, which was identified as EwS following the biopsy. The tumor, located in the thigh, was associated with infiltration of lymph nodes and blood vessels. After surgical resection in September 1978, the patient received adjuvant CHT according to ROSEN protocol (dactinomicin, doxorubicin, cyclophosphamide and vincristine) and adjuvant radiotherapy (RT). The doses of CHT were adjusted due to grade 3–4 leukopenia. The patient completed adjuvant treatment by September 1980. In April 1980, doxorubicin was withdrawn upon reaching the cumulative dose of 775 mg. Despite complications including pathological fractures, the patient showed no evidence of disease recurrence on imaging follow-up. Over the years, the patient underwent several surgical procedures, including a mastectomy for ductal carcinoma in situ and resection for a basal cell adenoma of the hard palate. Throughout this period, regular follow-up imaging showed no recurrence or metastatic disease. As of early 2025, the patient remains alive with no evidence of disease progression or recurrence, marking a 47-year survival. The long-term bone sarcoma survivors require vigilant, lifelong screening and care, as they are at risk of relapse, secondary neoplasms and pathological fractures, particularly during certain biological events, including pregnancy.

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Photo by National Cancer Institute on Unsplash

Photo by National Cancer Institute on Unsplash

Why is it important?

The prognosis for patients diagnosed with Ewing's sarcoma (EwS) substantially improved since the integration of chemotherapy (CHT) into treatment protocols in the early 1970s. In the preceding series, the 5-year overall survival rate was documented to be approximately 10%, while in the recent ones it ranges between 75% and 80%. Consequently, the reports of very long-term survivors (≥ 30 years post-treatment) are scarce, particularly in cases where secondary malignancies and pathological fractures are present. Therefore, we present the case of the longest (to our knowledge) survival in EwS reported to date. An 18-year-old female presented with a 10 × 7 × 7 cm tumor, which was identified as EwS following the biopsy. The tumor, located in the thigh, was associated with infiltration of lymph nodes and blood vessels. After surgical resection in September 1978, the patient received adjuvant CHT according to ROSEN protocol (dactinomicin, doxorubicin, cyclophosphamide and vincristine) and adjuvant radiotherapy (RT). The doses of CHT were adjusted due to grade 3–4 leukopenia. The patient completed adjuvant treatment by September 1980. In April 1980, doxorubicin was withdrawn upon reaching the cumulative dose of 775 mg. Despite complications including pathological fractures, the patient showed no evidence of disease recurrence on imaging follow-up. Over the years, the patient underwent several surgical procedures, including a mastectomy for ductal carcinoma in situ and resection for a basal cell adenoma of the hard palate. Throughout this period, regular follow-up imaging showed no recurrence or metastatic disease. As of early 2025, the patient remains alive with no evidence of disease progression or recurrence, marking a 47-year survival. The long-term bone sarcoma survivors require vigilant, lifelong screening and care, as they are at risk of relapse, secondary neoplasms and pathological fractures, particularly during certain biological events, including pregnancy.

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