Clinical activity of eribulin in advanced desmoplastic small round-cell tumor

Emambux, Sheik; Kind, Michèle; Loarer, François Le; Toulmonde, Maud; Stoeckle, Eberhard; Italiano, Antoîne

doi:10.1097/cad.0000000000000536

Cited by 11 publications

(12 citation statements)

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“…Less common salvage regimens include gemcitabine with docetaxel, cyclophosphamide with vinorelbine, and dacarbazine (8). Several other regimens have been trialed on an investigational basis and include carboplatin and actinomycin in addition to P6 drugs (5); pazopanib in heavily pre-treated patients (9, 10); vinorelbine, cyclophosphamide, and temsirolimus in patients with relapse (25); and eribulin in advanced cases (26). In a study in Germany by Sheet et al with the largest series of patients with DSRCT enrolled in prospective trials to date, the best outcome was observed with vincristine, dactinomycin, ifosfamide and doxorubicin (VAIA) (5).…”

Section: Discussionmentioning

confidence: 99%

Desmoplastic Small Round-cell Tumor: Retrospective Review of Institutional Data and Literature Review

et al. 2021

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Background: Desmoplastic small round-cell tumor (DSCRT) in adults is an extremely rare (age-adjusted incidence 0.3 per million) and aggressive sarcoma with limited data for optimal management. Patients and Methods: Retrospective analysis of patients with DSCRT diagnosis (2010DSCRT diagnosis ( -2020 was performed following Institutional Review Board approval. The follow-up period was from pathological diagnosis to the last patient contact. Endpoints were type of response and duration of response. Results: In the current analysis, first-line treatment in all cases was vincristine, anthracycline, and cyclophosphamide alternating with ifosfamide and etoposide (VAC-IE) with 100% response for a mean duration of 9.8 (range=5-12) months. Patients received 1-4 subsequent lines of therapy. All patients received temozolomide with irinotecan (50% partial response, duration 8-9 months). Two patients that underwent consolidative cytoreductive surgery with hyperthermic intraperitoneal chemotherapy had a longer survival (30.6 vs. 11.2 months). Patients suffered 100% mortality with a median survival was 17.8 (range=11.2-30.6) months. Conclusion: While aggressive multimodality treatment is always warranted for DSCRT, the options are limited by the multicentric presentation, short-lived initial response and lack of established subsequent therapy portending a poor prognosis. Consolidative cytoreductive surgery following first-line therapy may improve survival.Desmoplastic small round-cell tumor (DSCRT) in adults is a rare sarcoma predominantly affecting young adults, with an 3859 This article is freely accessible online.

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Section: Discussionmentioning

confidence: 99%

Desmoplastic Small Round-cell Tumor: Retrospective Review of Institutional Data and Literature Review

et al. 2021

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“…The following phase III study by the same group compared the efficacy of eribulin and dacarbazine in advanced LPS and leiomyosarcoma patients [ 25 ]. However, few data are available on eribulin activity in other aggressive sarcoma subtypes known for their refractoriness to chemotherapy [ 26 , 27 ].…”

Section: Introductionmentioning

confidence: 99%

Primary Culture of Undifferentiated Pleomorphic Sarcoma: Molecular Characterization and Response to Anticancer Agents

Vita

Recine

Mercatali

et al. 2017

IJMS

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Undifferentiated pleomorphic sarcoma (UPS) is an aggressive mesenchymal neoplasm with no specific line of differentiation. Eribulin, a novel synthetic microtubule inhibitor, has shown anticancer activity in several tumors, including soft tissue sarcomas (STS). We investigated the molecular biology of UPS, and the mechanisms of action of this innovative microtubule-depolymerizing drug. A primary culture from a patient with UPS was established and characterized in terms of gene expression. The activity of eribulin was also compared with that of other drugs currently used for STS treatment, including trabectedin. Finally, Western blot analysis was performed to better elucidate the activity of eribulin. Our results showed an upregulation of epithelial mesenchymal transition-related genes, and a downregulation of epithelial markers. Furthermore, genes involved in chemoresistance were upregulated. Pharmacological analysis confirmed limited sensitivity to chemotherapy. Interestingly, eribulin exhibited a similar activity to that of standard treatments. Molecular analysis revealed the expression of cell cycle arrest-related and pro-apoptotic-related proteins. These findings are suggestive of aggressive behavior in UPS. Furthermore, the identification of chemoresistance-related genes could facilitate the development of innovative drugs to improve patient outcome. Overall, the results from the present study furnish a rationale for elucidating the role of eribulin for the treatment of UPS.

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“…Esse tumor apresenta um perfil de imuno-histoquímica caracterizado pela coexpressão dos marcadores epiteliais (citoqueratina e antígeno de membrana epitelial), neural (enolase neurônio-específica e CD56), mesenquimal (vimentina), e miogênica (desmina), achados que convergiram para o diagnóstico de TDPCR deste relato 13,14 . Estudos recentes têm demonstrado uma associação entre o TDPCR e uma translocação (11,22)(p13; q12), que resulta em um gene de fusão entre os genes do sarcoma de Ewing e do tumor de Wilms 15 .…”

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“…O erlotinibe, um inibidor multialvo da tirosina quinase, levou à resposta parcial em um relato de caso 19 . A atividade contra a doença foi relatada com uso de pazopanibe 20,21 e eribulin 22 . Relato recente demonstrou resultados negativos com imatinibe 23 bevacizumabe também foi relatada [24][25][26] .…”

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Tumor Desmoplásico de Pequenas Células Redondas: Relato de Caso

Costa

Reis

Loureiro

et al. 2018

Rev. Brasileira.De.Cancerologia

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Introdução: O tumor desmoplásico de pequenas células redondas é uma rara neoplasia que se inicia e se espalha pela superfície peritoneal. Foi descrito pela primeira vez em 1989 e, em 1991, houve seu reconhecimento como entidade clínica e patológica distintas. Relato do caso: Homem de 34 anos apresentou quadro de dor abdominal e perda de peso, evoluindo para obstrução intestinal dois meses após. A laparotomia demonstrou grande massa abdominopélvica irressecável. O laudo anatomopatológico associado à imuno-histoquímica evidenciou diagnóstico de tumor desmoplásico de pequenas células redondas. A tomografia computadorizada confirmou derrame pleural bilateral, implantes peritoneais e massas abdominais e pélvicas. Realizou-se quimioterapia com carbo/taxol com intervalo de 21 dias. Substituiu-se o esquema para VAC/IE com intervalo de 21 dias, com resposta parcial, porém ainda se mantendo um tumor irressecável. Houve piora progressiva da performance do paciente, com evolução ao óbito por obstrução intestinal no 15º mês de seguimento. Conclusão: O tumor desmoplásico de pequenas células redondas, em razão da sua raridade, continua sendo um desafio para o diagnóstico e o tratamento.

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Clinical activity of eribulin in advanced desmoplastic small round-cell tumor

Cited by 11 publications

References 14 publications

Desmoplastic Small Round-cell Tumor: Retrospective Review of Institutional Data and Literature Review

Desmoplastic Small Round-cell Tumor: Retrospective Review of Institutional Data and Literature Review

Primary Culture of Undifferentiated Pleomorphic Sarcoma: Molecular Characterization and Response to Anticancer Agents

Tumor Desmoplásico de Pequenas Células Redondas: Relato de Caso

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