Single-Centre Experience of Systemic Treatment with Vincristine, Ifosfamide, and Doxorubicin Alternating with Etoposide, Ifosfamide, and Cisplatin in Adult Patients with Ewing Sarcoma

Abstract

The treatment of Ewing sarcoma (ES) in adult patients requires a multidisciplinary approach. Systemic therapy remains an important component of clinical management of this disease. ES is extremely rare in adult patients. Due to the rarity of the disease, no standard of care in terms of chemotherapy for the adult population exists, and the level of evidence for individual agents or some multidrug combinations is limited. Most regimens that are used in both adults and children include anthracyclines, etoposide, vincristine, cyclophosphamide, and ifosfamide. In this report, we describe our experience with the alternating use of triple combination therapies based on vincristine, ifosfamide, and doxorubicin (VIA) and an etoposide, ifosfamide, and cisplatin combination (VIP). We retrospectively evaluated the response rates, outcome, and tolerance of adult patients (n = 64) treated with VIA/VIP between 1990 and 2014. The patients included were treated with perioperative chemotherapy (53.1% neoadjuvant therapy and 17.2% adjuvant therapy) or had synchronous metastases at diagnosis (29.7%). Five-year overall survival rate was 52.2% for all patients, 72.2% for patients with localized disease, and 5.3% in patients with synchronous metastases. Overall response rate (ORR) was 37% after 2 cycles of VIA and 2 cycles of VIP. There were no patients with progressive disease (PD).

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“…The objective is to maximize the killing of cancer cells, minimize toxicity, delay the onset of drug resistance, and prolong survival, especially for sarcomas, where various alternative treatment regimens have been employed. For instance, in Ewing’s sarcoma, alternating triple combination therapies such as vincristine, ifosfamide, and doxorubicin (VIA) and etoposide, ifosfamide, and cisplatin (VIP) have been tested ( 43 ). Regimens for soft tissue sarcomas have utilized alternating cycles of cyclophosphamide, doxorubicin, and vincristine (VDC) and ifosfamide and etoposide (IE) chemotherapy ( 44 ).…”

Outcome of multidisciplinary treatment of peripheral primitive neuroectodermal tumor

“…However, severe acute toxicities were more frequent in the BuMel group, and the incidence of secondary malignancies in long-term survivors was not reported. Studies on other agents are more limited, thus no firm conclusion could be drawn at this stage [40][41][42][43] . To date, there is no standard treatment for refractory disease.…”

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“…The objective is to maximize the killing of cancer cells, minimize toxicity, delay the onset of drug resistance, and prolong survival, especially for sarcomas, where various alternative treatment regimens have been employed. For instance, in Ewing's sarcoma, alternating triple combination therapies such as vincristine, ifosfamide, and doxorubicin (VIA) and etoposide, ifosfamide, and cisplatin (VIP) have been tested (43). Regimens for soft tissue sarcomas have utilized alternating cycles of cyclophosphamide, doxorubicin, and vincristine (VDC) and ifosfamide and etoposide (IE) chemotherapy (44).…”