Pazopanib for treatment of typical solitary fibrous tumours: a multicentre, single-arm, phase 2 trial

Martin‐Broto, Javier; Cruz, Josefina; Penel, Nicolas; Cesne, Axel Le; Hindi, Nadia; Luna, Pablo; Moura, David S.; Bernabeu, Daniel; Álava, Enrique de; Löpez‐Guerrero, José Antonio; Dopazo, Joaquín; Peña‐Chilet, María; Gutiérrez, Antonio; Collini, Paola; Karanian, Marie; Redondo, Andrés; López‐Pousa, Antonio; Grignani, Giovanni; Díaz‐Martín, Juan; Marcilla, David; Fernández-Serra, Antonio; González-Aguilera, Cristina; Casali, Paolo G.; Blay, Jean‐Yves; Stacchiotti, Silvia

doi:10.1016/s1470-2045(19)30826-5

Cited by 54 publications

(60 citation statements)

References 27 publications

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“…37,38 Several tyrosine kinase inhibitors have antitumour activity in SFT, including sunitinib and pazopanib. [39][40][41] However, the clinical and radiological response varies considerably between patients, suggesting that different biological mechanisms might be activated in different tumours. Sunitinib's antitumour activity in SFT is mediated, at least partly, through a platelet derived growth factor receptor beta (PDGFRb)-dependent mechanism.…”

Section: Discussionmentioning

confidence: 99%

Clinical and molecular implications of NAB2-STAT6 fusion variants in solitary fibrous tumour

et al. 2021

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Solitary fibrous tumour (SFT) is a mesenchymal neoplasm characterised by pathognomonic NAB2-STAT6 gene fusions. The clinical implications and prognostic value of different fusion variants has not been clarified. In the current study, we explore the clinicopathological, prognostic and molecular differences between tumours with different fusions. Thirty-nine patients with localised, extrameningeal SFT were included, of whom 20 developed distant recurrence and 19 were without recurrence after long term follow-up. Capture-based RNA sequencing identified 12 breakpoint variants, which were categorised into two groups based on the STAT6 domain composition in the predicted chimeric proteins. Twenty-one of 34 (62%) sequenced tumours had fusions with most of the STAT6 domains intact and were classified as STAT6-Full. Thirteen tumours (38%) contained only the transactivation domain of STAT6 and were classified as STAT6-TAD. Tumours with STAT6-TAD fusions had a higher mitotic count (p=0.016) and were associated with inferior recurrence-free interval (p=0.004) and overall survival (p=0.012). Estimated 10year recurrence-free survival was 25% for patients with STAT6-TAD tumours compared to 78% for the STAT6-Full group. Distinct transcriptional signatures between the fusion groups were identified, including higher expression of FGF2 in the STAT6-TAD group and IGF2, EGR2, PDGFRB, STAT6 and several extracellular matrix genes in STAT6-Full tumours. In summary, we demonstrate that NAB2-STAT6 fusion variants are associated with distinct clinicopathological and molecular characteristics and have prognostic significance in extrameningeal SFT.

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Section: Discussionmentioning

confidence: 99%

Clinical and molecular implications of NAB2-STAT6 fusion variants in solitary fibrous tumour

et al. 2021

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“…M2 TAMs express CD163, CD115, and CD206; they are able to secrete IL-10, promote tumor growth, and foster angiogenesis. TAMs are crucial for many types of sarcoma but their potential role has been highlighted in leiomyosarcoma [108], and their prognostic value in solitary fibrous tumor [109]. Noteworthy is that TAMs are able to induce the CSCs phenotype and to support CSCs tumorigenic functions, thus representing attractive therapeutic targets.…”

Section: Targeting the Tumor Microenvironmentmentioning

confidence: 99%

Cancer Stem Cells in Soft-Tissue Sarcomas

Martínez-Delgado

Lacerenza

Obrador‐Hevia

et al. 2020

Cells

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Soft tissue sarcomas (STS) are a rare group of mesenchymal solid tumors with heterogeneous genetic profiles and clinical features. Systemic chemotherapy is the backbone treatment for advanced STS; however, STS frequently acquire resistance to standard therapies, which highlights the need to improve treatments and identify novel therapeutic targets. Increases in the knowledge of the molecular pathways that drive sarcomas have brought to light different molecular alterations that cause tumor initiation and progression. These findings have triggered a breakthrough of targeted therapies that are being assessed in clinical trials. Cancer stem cells (CSCs) exhibit mesenchymal stem cell (MSC) features and represent a subpopulation of tumor cells that play an important role in tumor progression, chemotherapy resistance, recurrence and metastasis. In fact, CSCs phenotypes have been identified in sarcomas, allied to drug resistance and tumorigenesis. Herein, we will review the published evidence of CSCs in STS, discussing the molecular characteristic of CSCs, the commonly used isolation techniques and the new possibilities of targeting CSCs as a way to improve STS treatment and consequently patient outcome.

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“…However, there is one reported case of a malignant SFT measuring only 3 cm in diameter (Table.2). Besides, Martin-Broto et al also suggested that overexpression of CD209 has also been related to poor prognosis in SFTs (1).…”

Section: Prognosismentioning

confidence: 99%

Two case reports of rare diseases occurring in rare parts: Splenic vein solitary fibrous tumor and Liver solitary fibrous tumor

Wang

Bao

et al. 2020

Preprint

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Background Solitary fibrous tumor (SFT) is a rare soft tissue tumor originating from mesenchyme. Two cases of SFT we report right now occurred in the splenic vein and liver respectively, this primary splenic vein SFT may be the first report case, and also the first report of liver recurrence SFT cured by orthotopic liver transplantation (OLT). Case presentation One case was a 37-year-old female patient whose primary tumor site was located in the splenic vein, which resulted in splenomegaly and hypersplenism; its recurrence again and again after surgical resection and eventually transferred to the liver, during 10 years of follow-up, 4 operations were performed, and he is in a good condition right now. The second case was a 54-year-old male patient whose primary tumor site was located in the liver, spleen and left side of the chest wall; however, he had no uncomfortable symptoms. Surgeons performed two operations to remove these tumors, totally. 6 years later, SFT recurrence in the liver, and given that the tumor was so large that it could not be completely surgical resected, we chose orthotopic liver transplantation (OLT), and no tumor recurrence during 12-month follow-up. Conclusion The reports of these two cases of SFT are very rare, especially the splenic vein SFT, which expand the understanding of SFT. The main treatment of SFT is still surgical resection, right now, and liver transplantation may be a new option treatment for the huge liver SFT.

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Pazopanib for treatment of typical solitary fibrous tumours: a multicentre, single-arm, phase 2 trial

Cited by 54 publications

References 27 publications

Clinical and molecular implications of NAB2-STAT6 fusion variants in solitary fibrous tumour

Clinical and molecular implications of NAB2-STAT6 fusion variants in solitary fibrous tumour

Cancer Stem Cells in Soft-Tissue Sarcomas

Two case reports of rare diseases occurring in rare parts: Splenic vein solitary fibrous tumor and Liver solitary fibrous tumor

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