Long-term Results of Therapy with Sunitinib in Metastatic Alveolar Soft Part Sarcoma

Jagodzińska-Mucha, Paulina; Świtaj, Tomasz; Kozak, Katarzyna; Koseła-Paterczyk, Hanna; Klimczak, Anna; Ługowska, Iwona; Rogala, Paweł; Wągrodzki, Michał; Falkowski, Sławomir; Rutkowski, Piotr

doi:10.5301/tj.5000617

Cited by 33 publications

(27 citation statements)

References 17 publications

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“…It is of note that stabilization of the disease was the most frequent response observed between 53% and 83% for patients treated with TKIs, including those receiving pazopanib in our study . This is not surprising in that antiangiogenic TKIs target the angiogenesis signaling pathways on the endothelial cells rather than tumor itself.…”

Section: Assessment Analysis and Discussionsupporting

confidence: 50%

A Phase II Trial of Pazopanib in Patients with Metastatic Alveolar Soft Part Sarcoma

et al. 2018

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Lessons Learned. Pazopanib shows a modest efficacy in metastatic alveolar soft part sarcoma.Clinical outcomes were comparable to those in previous studies using antiangiogenic drugs.Further prospective studies evaluating the benefit of pazopanib in alveolar soft part sarcoma with a larger sample are warranted to validate results.Background.Alveolar soft part sarcoma (ASPS) is a rare mesenchymal malignant tumor characterized by an unbalanced translocation, t(X;17)(p11.2;q25), which leads to the fusion of ASPSCR1 to the TFE3 transcription factor. Because this results in the upregulation of angiogenesis‐related transcripts, antiangiogenic drugs have been used in ASPS patients.Methods.This open‐label, single‐arm, multicenter, investigator‐initiated phase II trial was designed to evaluate efficacy and safety of pazopanib 800 mg once daily in patients with metastatic ASPS. The primary endpoint was investigator‐assessed overall response rate (ORR), and secondary endpoints were toxicity, progression‐free survival (PFS), and overall survival (OS). 68Ga‐RGD (Arg‐Gly‐Asp) positron emission tomography (PET) scan and gene expression profiling using NanoString platform were performed for biomarker analysis.Results.Six patients with histologically confirmed metastatic ASPS were enrolled between December 2013 and November 2014. Among six patients, one achieved a partial response (PR) (ORR 16.7%) and five patients showed stable disease (SD). With a median follow‐up of 33 months (range 18.7–39.3 months), median PFS was 5.5 months (95% confidence interval [CI] 3.4–7.6 months), and median OS was not reached. There were no severe toxicities except one patient with grade 3 diarrhea.Conclusion.Pazopanib showed modest antitumor activity with manageable toxicities for patients with metastatic ASPS.

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Section: Assessment Analysis and Discussionsupporting

confidence: 50%

A Phase II Trial of Pazopanib in Patients with Metastatic Alveolar Soft Part Sarcoma

et al. 2018

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“…Recently, agents such as cediranib, sunitinib, dasatinib, pazopanib, and tivantinib have been used in ASPS with promising results. In Table , we summarize the results of reports regarding targeted therapy for ASPS including the current series . It is difficult to assess clinical benefit in ASPS as the rate of tumor growth is variable, and patients may have prolonged periods of SD without any treatment.…”

Section: Discussionmentioning

confidence: 99%

Alveolar soft part sarcoma in children and young adults: A report of 69 cases

Flores

Harrison

Federman³

et al. 2018

Pediatric Blood & Cancer

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“…In both cases sunitinib retreatment lead to response to therapy [8]. These results were confirmed in a Polish single-centre group of patients treated in the Maria Sklodowska-Curie Institute -Oncology Center in Warsaw [9].…”

Section: Sunitinibmentioning

confidence: 57%

Advances in systemic treatment of advanced soft tissue sarcomas

Dudzisz-Śledź

Rogala

2019

Oncol Clin Pract

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Systemic treatment in soft tissue sarcoma (STS) is an important element of therapy in a disease eligible for combined treatment with a radical intention and is the basis for the treatment of an unresectable or metastatic disease. The possibilities of treating STS are limited and for many years progress in this area was minor and the main drugs used in this indication were and still remain anthracyclines and alkylating agents. Clinical trials with new drugs are difficult for STS due to the heterogeneity of these tumours and due to their rare occurrence. Over the past two decades, there have been tested many substances in this indication, including molecularly targeted drugs. Great success was imatinib in the treatment of gastrointestinal stromal tumours (GIST). Other drugs in STS have been tested and approved for use, e.g. sunitinib and regorafenib in the treatment of GIST, pazopanib in the treatment of non-GIST STS, trabectedin and olaratumab in the treatment of STS. First reports on the effectiveness of immunotherapy in the treatment of rare subtypes of STS are also available.

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Long-term Results of Therapy with Sunitinib in Metastatic Alveolar Soft Part Sarcoma

Abstract: Our analysis confirms the long-term efficacy of sunitinib in patients with advanced ASPS.

Cited by 33 publications

References 17 publications

A Phase II Trial of Pazopanib in Patients with Metastatic Alveolar Soft Part Sarcoma

A Phase II Trial of Pazopanib in Patients with Metastatic Alveolar Soft Part Sarcoma

Alveolar soft part sarcoma in children and young adults: A report of 69 cases

Advances in systemic treatment of advanced soft tissue sarcomas

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