Durable clinical activity of single-agent bevacizumab in a nonagenarian patient with metastatic alveolar soft part sarcoma

2012 ◽  
Vol 23 (7) ◽  
pp. 745-748 ◽  
Author(s):  
Olivier Mir ◽  
Pascaline Boudou-Rouquette ◽  
Frédérique Larousserie ◽  
Benoit Blanchet ◽  
Antoine Babinet ◽  
...  
Keyword(s):  
Soft Part ◽  
Single Agent ◽  
Alveolar Soft Part Sarcoma ◽  
Clinical Activity

scholarly journals Cediranib for Metastatic Alveolar Soft Part Sarcoma

2013 ◽  
Vol 31 (18) ◽  
pp. 2296-2302 ◽  
Author(s):  
Shivaani Kummar ◽  
Deborah Allen ◽  
Anne Monks ◽  
Eric C. Polley ◽  
Curtis D. Hose ◽  
...  
Keyword(s):  
Partial Response ◽  
Disease Control ◽  
Phase Ii ◽  
Stable Disease ◽  
Soft Part ◽  
Expression Profiles ◽  
Single Agent ◽  
Disease Control Rate ◽  
Alveolar Soft Part Sarcoma ◽  
Tumor Biopsies

Purpose Alveolar soft part sarcoma (ASPS) is a rare, highly vascular tumor, for which no effective standard systemic treatment exists for patients with unresectable disease. Cediranib is a potent, oral small-molecule inhibitor of all three vascular endothelial growth factor receptors (VEGFRs). Patients and Methods We conducted a phase II trial of once-daily cediranib (30 mg) given in 28-day cycles for patients with metastatic, unresectable ASPS to determine the objective response rate (ORR). We also compared gene expression profiles in pre- and post-treatment tumor biopsies and evaluated the effect of cediranib on tumor proliferation and angiogenesis using positron emission tomography and dynamic contrast-enhanced magnetic resonance imaging. Results Of 46 patients enrolled, 43 were evaluable for response at the time of analysis. The ORR was 35%, with 15 of 43 patients achieving a partial response. Twenty-six patients (60%) had stable disease as the best response, with a disease control rate (partial response + stable disease) at 24 weeks of 84%. Microarray analysis with validation by quantitative real-time polymerase chain reaction on paired tumor biopsies from eight patients demonstrated downregulation of genes related to vasculogenesis. Conclusion In this largest prospective trial to date of systemic therapy for metastatic ASPS, we observed that cediranib has substantial single-agent activity, producing an ORR of 35% and a disease control rate of 84% at 24 weeks. On the basis of these results, an open-label, multicenter, randomized phase II registration trial is currently being conducted for patients with metastatic ASPS comparing cediranib with another VEGFR inhibitor, sunitinib.

High clinical activity of pembrolizumab in chordoma, alveolar soft part sarcoma (ASPS) and other rare sarcoma histotypes: The French AcSé pembrolizumab study from Unicancer.

2021 ◽  
Vol 39 (15_suppl) ◽  
pp. 11520-11520
Author(s):  
Jean-Yves Blay ◽  
Nicolas Penel ◽  
Isabelle Laure Ray-Coquard ◽  
Sophie Cousin ◽  
Francois Bertucci ◽  
...  
Keyword(s):  
Response Rate ◽  
Soft Part ◽  
Objective Response ◽  
Alveolar Soft Part Sarcoma ◽  
Clinical Activity ◽  
Tumor Type ◽  
Malignant Rhabdoid Tumor ◽  
Multicentric Study ◽  
Best Response ◽  
Duration Of Response

11520 Background: AcSé Pembrolizumab is a Phase 2, non-randomized parallel arms, open-label, multicentric study from Unicancer investigating the efficacy and safety of pembrolizumab monotherapy in different cohorts of patients with rare cancers (NCT03012620). Here we report the results of pembrolizumab in the rare sarcoma cohort. Methods: Selected histotypes were all rare sarcomas patients (pts) (incidence < 0.2/100,000/year). Main inclusion criteria were age > 18, ECOG PS≤1 and advanced or metastatic disease resistant to standard treatment. Patients received pembrolizumab 200 mg IV as a 30-minute infusion on Day 1 of every 21-day cycle for a maximum of 2 years. The primary endpoint was the confirmed objective response rate according to RECIST v1.1 at 12 weeks. Secondary endpoints included clinical benefit rate, duration of response, progression-free survival (PFS), overall survival (OS), and safety. Five groups of pts were distinguished, namely chordoma, alveolar soft-part sarcoma (ASPS), desmoplastic small round cell tumor (DSRCT), smarca4 deficient malignant rhabdoid tumor (SMRT), and other histotypes. Results: 98 patients including 34 with chordoma, 14 ASPS, 11 SMRT, 8 DSCRT and 31 with other histotypes, were included from July 2017 to December 2020. The median number of cycles was 5 (range, 1 to 35) with 78 (79.6%) patients who discontinued the trial after a median of 4 cycles. There were 6 (7.3%) partial response (PR) at 12 weeks. The best response was CR in 1 patient (1%), PR in 14 patients (14.3%), and stable disease (SD) in 33 (33.7%). Median duration of response was 8.2 months [IQR, 4.1 to 9.0]. The occurrence of best response depended on the histotype, with 3 (8.8%) responses in chordoma, 7 (50%) in ASPS, 3 (27%) in SMRT, 1 (12.5%) in DSCRT and 1 (3.2%) in other histotypes (p = 0.0011). At the data cut off, median PFS was 2.75 months, and median OS was 19.7 months on the overall population. Outcomes differed according to the histotype group, with the 12 months PFS rates at 31.2% (chordoma), 35.7% (ASPS), 18.2% (SMRT), 0% (DSCRT) and 3.3% (other), respectively (p < 0.0001), and median PFS at 6.6 (chordoma), 7.5 (ASPS), 1.1 (SMRT), 2.1 (DSCRT) and 2.1 months (other), while 1-year OS rates were 76.6% (chordoma), 85.7% (ASPS), 36.4% (SMRT), 17.5% (DSCRT) and 42.9% (other) with median OS only reached for SMRT (2.4 months), DSRCT (10 months), and the other histotype group (7.1 months) (p = 0.004). The side effect profile of pembrolizumab was similar to other tumor type. Conclusions: Pembrolizumab is safe and well tolerate in this pop od STS pts, AcSé study reports high levels response rate and prolonged activity in selected subtypes of rare sarcomas. Clinical trial information: NCT03012620.

scholarly journals Biomarkers in Hepatobiliary Cancers: What is Useful in Clinical Practice?

Cancers ◽  
2021 ◽  
Vol 13 (11) ◽  
pp. 2708
Author(s):  
Alice Boilève ◽  
Marc Hilmi ◽  
Matthieu Delaye ◽  
Annemilaï Tijeras-Raballand ◽  
Cindy Neuzillet
Keyword(s):  
Clinical Practice ◽  
Single Agent ◽  
Survival Rates ◽  
Critical Evaluation ◽  
Treatment Modalities ◽  
Molecular Profile ◽  
Clinical Activity ◽  
Liver Malignancies ◽  
Antiangiogenic Drugs ◽  
Systemic Therapies

Hepatocellular carcinoma (HCC) and biliary tract cancers (BTC) exhibit a poor prognosis with 5-year overall survival rates around 15%, all stages combined. Most of these primary liver malignancies are metastatic at diagnostic, with only limited therapeutic options, relying mainly on systemic therapies. Treatment modalities are different yet partially overlapping between HCC and BTC. The complex molecular profile of BTC yields to several actionable therapeutic targets, contrary to HCC that remains the field of antiangiogenic drugs in non-molecularly selected patients. Immunotherapy is now validated in the first line in HCC in combination with bevacizumab, while clinical activity of single agent immunotherapy appears limited to a subset of patients in BTC, still poorly characterized, and combinations are currently under investigation. In this review, we provide a critical evaluation and grading of clinical relevance on (i) the main prognostic biomarkers in HCC and BTC, (ii) the main theragnostic biomarkers in both tumors, and lastly (iii) what is recommended in clinical practice.

Alveolar soft-part sarcoma

1990 ◽  
Vol 26 (4) ◽  
pp. 783
Author(s):  
S H Jung ◽  
D W Sung ◽  
Y Yoon ◽  
W S Choi ◽  
J H Lim ◽  
...  
Keyword(s):  
Soft Part ◽  
Alveolar Soft Part Sarcoma

scholarly journals Dual-color, Break-apart FISH Assay on Paraffin-embedded Tissues as an Adjunct to Diagnosis of Xp11 Translocation Renal Cell Carcinoma and Alveolar Soft Part Sarcoma

2010 ◽  
Vol 34 (6) ◽  
pp. 757-766 ◽  
Author(s):  
Minghao Zhong ◽  
Patricia De Angelo ◽  
Lisa Osborne ◽  
Megan Keane-Tarchichi ◽  
Michael Goldfischer ◽  
...  
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Soft Part ◽  
Alveolar Soft Part Sarcoma ◽  
Dual Color

Alveolar soft part sarcoma of the oral and maxillofacial region: clinical analysis in a series of 18 patients

2015 ◽  
Vol 119 (4) ◽  
pp. 396-401 ◽  
Author(s):  
Hong-Wei Wang ◽  
Xing-Jun Qin ◽  
Wen-Jun Yang ◽  
Li-Qun Xu ◽  
Tong Ji ◽  
...  
Keyword(s):  
Soft Part ◽  
Clinical Analysis ◽  
Alveolar Soft Part Sarcoma ◽  
Maxillofacial Region ◽  
Oral And Maxillofacial Region

Tc-99m HMDP Accumulation in an Alveolar Soft Part Sarcoma

1993 ◽  
Vol 18 (7) ◽  
pp. 615 ◽  
Author(s):  
TSUNEO HIRANO ◽  
HIDENORI OHTAKE ◽  
KEIGO ENDO ◽  
AKIHIRO NAKAMUR ◽  
YASUHITO SASAKI
Keyword(s):  
Soft Part ◽  
Alveolar Soft Part Sarcoma
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