A single institution experience of combined modality management of extra skeletal Ewings sarcoma

Abstract

Background: Extraskeletal Ewings sarcoma are rare tumors for which there is no consensus on optimal management.

Multimodality imaging features, metastatic pattern and clinical outcome in adult extraskeletal Ewing sarcoma: experience in 26 patients

“…Aggressive management with multimodality treatment is recommended to improve survival rates. 18,20 The high incidence of local recurrence in earlier series was owing to the lack of post-operative radiotherapy. 3 The 5-year survival in the study by El Weshi et al 3 was 46% with 35 deaths out of 57 patients.…”

A case report on retroperitoneal PNET/EWS

“…Local adjuvant radiotherapy after complete resection cannot significantly reduce the local recurrence because of high doses and side effects that require stringent protective measures [12,15]. Venkitaraman et al [16] proposed that intensive chemotherapy is necessary after complete resection of the tumor or radiotherapy with doses >50 Gy, for patients with PNET/ EWS sarcoma outside bone in order to obtain good effect. Of 14 PNET/EWS patients without metastases who underwent combinational chemotherapy (5 with VAC procedures, 5 with VACA procedures, four with VAC/IE procedures), 8 were cured, 2 partially cured, and 4 had progressive deterioration [16].…”

“…Venkitaraman et al [16] believed that high hemoglo-bin, low lactate dehydrogenase, VAC/IE chemotherapy, and complete response to chemotherapy are the factors for raising tumor-free survival rate and overall survival rate. Martin et al [15] considered the initial performance of tumor (primary or recurrent) is an important prognostic factor, whereas tumor location, size, and age make no statistical significance [15,16]. PNET/EWS patients without metastatic who received combination therapy had 5-year survival rate of 60% and overall survival rate of 30% [16].…”

“…Martin et al [15] considered the initial performance of tumor (primary or recurrent) is an important prognostic factor, whereas tumor location, size, and age make no statistical significance [15,16]. PNET/EWS patients without metastatic who received combination therapy had 5-year survival rate of 60% and overall survival rate of 30% [16]. Aggressive PNET/EWS causes distant metastases at early stages, frequent local recurrence and regional lymph nodes, lungs, liver, bone and bone marrow metastasis, and has poor prognosis with the median survival time of about two years; the leading cause of death is metastasis to the lung, bone, liver and other organs.…”

Primary Ewing's Sarcoma/Primitive Neuroectodermal Tumor of Kidney with Caval Involvement in a Pregnant Woman

“…Ewing's family of tumors arise primarily from bones and they are rarely of extraskeletal origin [2,3]. It is most commonly found as a mass in the axial skeleton and in the soft tissue mass in the trunk.…”