Long-lasting multiagent chemotherapy in adult high-risk Ewing’s sarcoma of bone

Purpose The European Intergroup Cooperative Ewing's Sarcoma Study investigated whether cyclophosphamide has a similar efficacy as ifosfamide in standard-risk (SR) patients and whether the addition of etoposide improves survival in high-risk (HR) patients. Patients and Methods SR patients (localized tumors, volume <100 mL) were randomly assigned to receive four courses of vincristine, dactinomycin, ifosfamide, and doxorubicin (VAIA) induction therapy followed by 10 courses of either VAIA or vincristine, dactinomycin, cyclophosphamide, and doxorubicin (VACA; cyclophosphamide replacing ifosfamide). HR patients (volume ≥100 mL or metastases) were randomly assigned to receive 14 courses of either VAIA or VAIA plus etoposide (EVAIA). Outcome measures were event-free survival (EFS; defined as the time to first recurrence, progression, second malignancy, or death) and overall survival (OS). Results A total of 647 patients were randomly assigned: 79 SR patients were assigned to VAIA, 76 SR patients were assigned to VACA, 240 HR were assigned to VAIA, and 252 HR patients were assigned to EVAIA. The median follow-up was 8.5 years. In the SR group, the hazard ratios (VACA v VAIA) for EFS and OS were 0.91 (95% CI, 0.55 to 1.53) and 1.08 (95% CI, 0.58 to 2.03), respectively. There was a higher incidence of hematologic toxicities in the VACA arm. In the HR group, the EFS and OS hazard ratios (EVAIA v VAIA) indicated a 17% reduction in the risk of an event (95% CI, −35% to 5%; P = .12) and 15% reduction in dying (95% CI, −34% to 10%), respectively. The effect seemed greater among patients without metastases (hazard ratio = 0.79; P = .16) than among those with metastases (hazard ratio = 0.96; P = .84). Conclusion Cyclophosphamide seemed to have a similar effect on EFS and OS as ifosfamide in SR patients but was associated with increased toxicity. In HR patients, the addition of etoposide seemed to be beneficial.

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Ewing's sarcoma with distant metastasis: A brief note on management and emerging therapies

Clinics and Practice ◽

10.4081/cp.2019.1111 ◽

2019 ◽

Vol 9 (3) ◽

Author(s):

Girish Gulab Meshram ◽

Neeraj Kaur ◽

Kanwaljeet Singh Hura

Keyword(s):

High Risk ◽

Distant Metastasis ◽

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Molecular Targets ◽

Chemotherapeutic Agents ◽

Young Population ◽

Emerging Therapies ◽

The Right ◽

High Risk Case

Ewing’s sarcoma is an aggressive fatal malignancy of bones and soft-tissue. It predominantly affects the young population, with a worldwide incidence of three cases per million. The pelvis, extremities, and ribs are the most common sites. We present a case of massive Ewing’s sarcoma of the right femur with metastasis to bones and lungs. The patient was treated with chemotherapy. However, he succumbed to his illness before completion of therapy. In conclusion, Ewing’s sarcoma with distant metastasis is a high risk case with poor prognosis. Integrating novel molecular targets with conventional chemotherapeutic agents holds a promise for high-risk Ewing’s sarcoma patients.

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Faculty Opinions recommendation of Results of the EICESS-92 Study: two randomized trials of Ewing's sarcoma treatment--cyclophosphamide compared with ifosfamide in standard-risk patients and assessment of benefit of etoposide added to standard treatment in high-risk patients.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.1123254.580394 ◽

2008 ◽

Author(s):

Stephen Lessnick

Keyword(s):

High Risk ◽

Randomized Trials ◽

Standard Treatment ◽

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

High Risk Patients ◽

Risk Patients ◽

Sarcoma Treatment ◽

Standard Risk

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Ewing’s sarcoma/primitive neuroectodermal tumor of the prostate a case report and literature review

Canadian Urological Association Journal ◽

10.5489/cuaj.1394 ◽

2013 ◽

Vol 7 (5-6) ◽

Author(s):

Tao Wu ◽

Tao Jin ◽

Deyi Luo ◽

Lin Chen ◽

Xiang Li

Keyword(s):

Primitive Neuroectodermal Tumor ◽

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Transrectal Ultrasound ◽

Neuroectodermal Tumor ◽

Resonance Imaging ◽

Mri Scan ◽

Difficult Defecation ◽

Magnetic Resonance Imaging Mri ◽

Multiagent Chemotherapy

We present a case of Ewing’s sarcoma (ES) and primitive neuroectodermal tumor (PNET) of prostate. A 29-year-old male presented with difficult defecation and anus distention and was found on magnetic resonance imaging (MRI) scan of the pelvis to have a prostate tumor. A transrectal ultrasound guided needle biopsy confirmed the diagnosis. The patient underwent cystoprostatectomy and replacement ileocystoplasty and was followed by multiagent chemotherapy. PNET/EWS of the prostate is an extremely rare neoplasm. And the prognosis is very poor, so we should pay enough attention to the differential diagnosis and treatment.

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Ewing’s Sarcoma of the Mandible-A Rare Case Report

JMS SKIMS ◽

10.33883/jms.v14i2.90 ◽

2011 ◽

Vol 14 (2) ◽

pp. 68-70

Author(s):

Shahid Hassan ◽

BH Sripathi Rao ◽

Gunachandra Rai

Keyword(s):

Rare Case ◽

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Radiographic Examination ◽

Neural Tissues ◽

Rare Case Report ◽

Radiolucent Lesion ◽

Children And Young Adults ◽

Multiagent Chemotherapy ◽

Ewing’S Tumor

Ewing’s sarcoma is a malignant tumor of bones that primarily affects children and young adults. The true origin of this small round cell lesion still remains controversial.It was originally described by James Ewing in 1921 as arising from undifferentiated osseous mesenchymal cells, however, recent studies suggests that Ewing’s tumor might be neuroectodermally derived from various degrees of differentiation of the primitive neural tissues. This paper reports a rare case of ES of the mandible in a 11-year-old girl, which had been previously misdiagnosed and treated as a dental abscess. In the clinical examination, a hard immobile expansive mass of 2cm diameter was observed on the left side of the mandible. Radiographic examination revealed a diffuse radiolucent lesion with ill-defined borders and wide vestibular bone plate destruction. Microscopically, the tumor was composed by monotonous small round cells that exhibited immunoreactivity for CD99, vimentin and desmin. Surgical resection of mandible followed by mandibular reconstruction was adopted. The patient was subjected to multiagent chemotherapy with Vincristine [VC],Dactinomycin [AC],Cyclophosphamide [CP] and Doxorubicin [AD]). JMS 2011;14(2):68-70

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PP-077 TREATMENT OF HIGH RISK EWING’S SARCOMA WITH HDC AND SCT

Leukemia Research ◽

10.1016/s0145-2126(14)70131-6 ◽

2014 ◽

Vol 38 ◽

pp. S50-S51

Author(s):

S. Ozaydın ◽

M. Ozturk ◽

N. Karadurmus ◽

G. Erdem ◽

F. Arpacı

Keyword(s):

High Risk ◽

Ewing’S Sarcoma ◽

Ewing's Sarcoma

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Ewing's sarcoma of soft tissues in childhood: a report from the Intergroup Rhabdomyosarcoma Study, 1972 to 1991.

Journal of Clinical Oncology ◽

10.1200/jco.1997.15.2.574 ◽

1997 ◽

Vol 15 (2) ◽

pp. 574-582 ◽

Cited By ~ 150

Author(s):

R B Raney ◽

L Asmar ◽

W A Newton ◽

C Bagwell ◽

J C Breneman ◽

...

Keyword(s):

Soft Tissues ◽

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Distant Metastases ◽

Residual Tumor ◽

Complete Response ◽

Left Behind ◽

Multiagent Chemotherapy ◽

Extraosseous Ewing’S Sarcoma

PURPOSE One hundred thirty of 2,792 patients (5%) registered on three Intergroup Rhabdomyosarcoma Study clinical trials (IRS-I, -II, and -III) from 1972 to 1991 had an extraosseous Ewing's sarcoma (EOE). We report here the results of multimodality therapy for this tumor. PATIENTS AND METHODS The 130 patients were less than 21 years of age; 70 (54%) were males. Primary tumor sites were on the trunk in 41 patients, an extremity in 34, the head/neck in 23, the retroperitoneum/pelvis in 21, and other sites in 11. One hundred fourteen patients had no metastases at diagnosis. In 21 patients, the tumor was completely resected; in 30, the localized or regional tumor was grossly resected, and in 63 patients, grossly visible sarcoma was left behind. Sixteen patients (12%) had distant metastases at diagnosis. All patients were given multiagent chemotherapy and most received irradiation (XRT); none were treated with bone marrow transplantation. RESULTS One hundred seven patients (82%) achieved a complete response. At 10 years, 62%, 61%, and 77% of the patients were alive after treatment on IRS-I, IRS-II, or IRS-III therapeutic protocols, respectively, similar to figures obtained in all IRS patients. At last follow-up evaluation, 42 patients had died of progressive tumor and one of infection. Survival at 10 years was most likely for patients with tumor that arose in the head and neck, extremities, and trunk, and for those who underwent grossly complete tumor removal before initiation of chemotherapy. For patients with localized, gross residual tumor, adding doxorubicin (DOX) to the combination of vincristine, dactinomycin, cyclophosphamide (VAC), and XRT did not significantly improve survival in 39 patients (62% alive at 10 years) compared with that of 24 patients treated with VAC and XRT without DOX (65% alive at 10 years, P = .93). CONCLUSION This series indicated that EOE in children is similar to rhabdomyosarcoma (RMS) in its response to multimodal treatment. No benefit was apparent from the addition of DOX to VAC chemotherapy in patients with gross residual EOE.

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High-risk Ewing's sarcoma: End intensification using bone marrow transplantation

International Journal of Radiation Oncology*Biology*Physics ◽

10.1016/0360-3016(87)91190-4 ◽

1987 ◽

Vol 13 ◽

pp. 181-182 ◽

Cited By ~ 1

Author(s):

Robert B. Marcus ◽

John R. Graham-Pole ◽

Dempsey S. Springfield ◽

Samuel Grow ◽

Nancy P. Mendenhall ◽

...

Keyword(s):

Bone Marrow ◽

Bone Marrow Transplantation ◽

High Risk ◽

Marrow Transplantation ◽

Ewing’S Sarcoma ◽

Ewing's Sarcoma

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Primary Ewing’s sarcoma of the temporal bone: a rare entity and review of the literature

BMJ Case Reports ◽

10.1136/bcr-2019-230768 ◽

2019 ◽

Vol 12 (10) ◽

pp. e230768

Author(s):

Jeewan Ram Vishnoi ◽

Vijay Kumar ◽

Kirti Srivastava ◽

Sanjeev Misra

Keyword(s):

Temporal Bone ◽

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Bone Tumour ◽

Rare Entity ◽

Review Of The Literature ◽

Radiological Features ◽

Primary Bone Tumour ◽

Primary Bone ◽

Multiagent Chemotherapy

Ewing’s sarcoma (ES) is the second most common malignant primary bone tumour in children and adolescents. It primarily affects the diaphysis of long bones and pelvis. ES arising from temporal bone is extremely rare. To date, 43 such cases have been described in the literature. Clinical and radiological features are non-specific. Diagnosis is based mainly on immunohistochemistry. The present article presents an extremely rare case of ES of the temporal bone in a 20-year young man, and he was successfully treated with multiagent chemotherapy and radiotherapy.

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