Chemotherapy in Ewing’s sarcoma

Jain, Sandeep; Kapoor, Gauri

doi:10.4103/0019-5413.69305

Cited by 38 publications

(20 citation statements)

References 55 publications

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“…Experience from the pre-chemotherapy era taught us that resection of the primary tumor-mass alone does not prohibit disease progression, and almost every EwS patient will develop metastases during the course of disease in absence of any systemic therapy. Thus, it is assumed that micrometastatic disease is generally present at diagnosis, but chemotherapy is able to eradicate disseminated tumor cells in the approximately 70% of patients lacking evidence of metastases at diagnosis [ 307 ]. This may imply that, at diagnosis, it is the high overall tumor burden, which makes the difference for patient survival in primary metastatic disease.…”

Section: Scientific Perspectives On Clinical Enigmas Of Disseminated Ews Diseasementioning

confidence: 99%

Ewing Sarcoma—Diagnosis, Treatment, Clinical Challenges and Future Perspectives

Zöllner

Amatruda

Bauer

et al. 2021

JCM

148

114

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Ewing sarcoma, a highly aggressive bone and soft-tissue cancer, is considered a prime example of the paradigms of a translocation-positive sarcoma: a genetically rather simple disease with a specific and neomorphic-potential therapeutic target, whose oncogenic role was irrefutably defined decades ago. This is a disease that by definition has micrometastatic disease at diagnosis and a dismal prognosis for patients with macrometastatic or recurrent disease. International collaborations have defined the current standard of care in prospective studies, delivering multiple cycles of systemic therapy combined with local treatment; both are associated with significant morbidity that may result in strong psychological and physical burden for survivors. Nevertheless, the combination of non-directed chemotherapeutics and ever-evolving local modalities nowadays achieve a realistic chance of cure for the majority of patients with Ewing sarcoma. In this review, we focus on the current standard of diagnosis and treatment while attempting to answer some of the most pressing questions in clinical practice. In addition, this review provides scientific answers to clinical phenomena and occasionally defines the resulting translational studies needed to overcome the hurdle of treatment-associated morbidities and, most importantly, non-survival.

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Section: Scientific Perspectives On Clinical Enigmas Of Disseminated Ews Diseasementioning

confidence: 99%

Ewing Sarcoma—Diagnosis, Treatment, Clinical Challenges and Future Perspectives

Zöllner

Amatruda

Bauer

et al. 2021

JCM

148

114

View full text Add to dashboard Cite

show abstract

“…It is reported that patients with metastatic disease show poor outcomes and when metastasis spread to bone and bone marrow, the prognosis will be even worse [ 41 ]. Unfortunately, patients with metastatic disease often present resistance to chemotherapeutic agents [ 42 ], but the clinical data demonstrated that even localized tumour can relapse. Interestingly, the investigation of β3-ARs expression on CD45 − CD99 + cells showed that the presence of a high or low β3-ARs level could be useful to further separate into subgroups.…”

Section: Discussionmentioning

confidence: 99%

Preliminary Study on β3-Adrenoreceptor as Predictor Marker of Relapse in Ewing Sarcoma Patients

et al. 2020

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Ewing sarcoma (EWS) is a paediatric aggressive malignant tumour of bones and soft tissues. Multidisciplinary chemotherapies, surgical resection, and radiation represent the only strategies counteracting the disease, however spreading and relapse of disease still remain a clinical issue. Circulating tumour cells (CTCs) are an important feature of EWS but the prognostic significance has not been, yet, clarified. CTCs have been found both in patients with localized disease and in those who recur or metastasize. The identification of markers that can detect recurrences and metastasis remains an important challenge for research. Unfortunately, even most of patients with localized cancer relapsed and the reason has not yet been fully understood. In this clinical study on EWS patients, we evaluated the expression of CD99 antigen and beta-3 adrenergic receptor (β3-AR) on CTCs and bioptic derived cells by flow cytometry. The preliminary data revealed a higher β3-AR expression on cells derived from metastatic or relapsed patients, suggesting a role for the β3-AR as a possible predictive maker of disease recurrence in both patients with metastatic and localized disease.

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“…Prior to the advent of modern chemotherapy, <10% of patients with EFT survived beyond five years following diagnosis ( 14 ). The Intergroup Ewing’s Sarcoma Study and Cooperative Ewing Sarcoma Study recommend the standard chemotherapy comprising between four and six drugs, including doxorubicin, cyclophosphamide, vincristine, ifosfamide, etoposide and actinomycin ( 15 ). However, a standard therapy for recurrent tumors has not been established and autologous peripheral blood stem cell transfusion following chemotherapy is occasionally reported ( 16 ).…”

Section: Discussionmentioning

confidence: 99%

Primitive neuroectodermal tumor originating in the vulva: A case report

Matsuda

Ichimura

Kasai³

et al. 2014

Oncology Letters

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Primitive neuroectodermal tumors (PNETs) exhibit chromosomal translocations in common with those of Ewing’s sarcoma. They usually originate in bone or soft tissue but rarely arise in the vulva. The current case report presents a case of PNET originating in the vulva in a 60-year-old female, who previously underwent enucleation of a vulvar tumor in another hospital. The pathologist suspected a histopathological diagnosis of PNET, and simple vulvectomy and resection of the inguinal lymph nodes were performed. An ~3 cm mass recurred in the right side of the vulva four years following the initial surgery and the tumor was excised. The tumor comprised small, round-to-oval nuclei and stained positively for MIC-2, synaptophysin, neuron-specific enolase and neurofilament antibodies. To date, the patient remains alive and with no evidence of disease four years following multidisciplinary treatment, despite PNETs usually exhibiting a poor prognosis. This is due to the small tumor size and the absence of distant metastasis.

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Chemotherapy in Ewing’s sarcoma

Cited by 38 publications

References 55 publications

Ewing Sarcoma—Diagnosis, Treatment, Clinical Challenges and Future Perspectives

Ewing Sarcoma—Diagnosis, Treatment, Clinical Challenges and Future Perspectives

Preliminary Study on β3-Adrenoreceptor as Predictor Marker of Relapse in Ewing Sarcoma Patients

Primitive neuroectodermal tumor originating in the vulva: A case report

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