Pazopanib efficacy in recurrent central nervous system hemangiopericytomas

Apra, Caroline; Alentorn, Agustí; Mokhtari, Karima; Kalamaridès, Michel; Sanson, Marc

doi:10.1007/s11060-018-2870-0

Cited by 12 publications

(6 citation statements)

References 17 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Our ndings, associated with the histological identi cation of hemangiopericytomas with CNS-SFT and with previously published molecular data [5], suggests a common therapeutic approach for all types of SFT, regardless of their localization, might be relevant. As already reported in some cases [42], treatments usually used in soft tissues SFT have proved useful in meningeal SFT and could be discussed with general oncologists, rather than using meningiomas treatments in CNS-SFT, with this in mind that the treatment of meningeal localizations may have to be adapted for adequate delivery.…”

Section: Discussionmentioning

confidence: 97%

Molecular description of meningeal solitary fibrous tumors/hemangiopericytomas compared to meningiomas: two completely separate entities

Apra¹,

Guillemot

Bouvier

et al. 2021

Preprint

Self Cite

View full text Add to dashboard Cite

Introduction: Meningeal solitary fibrous tumors (SFT), like all SFT, are defined by NAB2-STAT6 fusion and share clinicopathologic similarities with meningiomas, the most frequent meningeal tumors. Our aim is to establish the molecular identity of meningeal SFT and seek molecular prognostic factors. Methods: RNA sequencing and whole exome sequencing (WES) were performed in STAT6-positive SFT and grade 2–3 meningiomas, and data concerning other soft tissues tumors was obtained from the local database. Uniform manifold approximation and projection (UMAP), individual gene expression and Gene Set Enrichment Analysis (GSEA) were performed. Results: RNA clustering shows that SFT share a common molecular signature, different from any other type of tumoral tissue. Meningeal SFT aggregate with other SFT, with no clinical or histological subgroup. Comparison of genes expressions suggests significant over-expressions of ZIC2, ZIC3, ZIC5, GABBR2, TP53 in CNS-SFT. The pathogenic TP53 c.743G > T variant, previously undescribed in SFT, was found in one sample of meningeal SFT during malignant progression. Conclusions: Meningeal SFT are molecular counterparts of extra-meningeal SFT, completely separate from meningiomas. They might develop from the same tissues and benefit from the same treatments as SFT.

show abstract

Section: Discussionmentioning

confidence: 97%

Molecular description of meningeal solitary fibrous tumors/hemangiopericytomas compared to meningiomas: two completely separate entities

Apra¹,

Guillemot

Bouvier

et al. 2021

Preprint

Self Cite

View full text Add to dashboard Cite

show abstract

“…Stereotactic radiosurgery has been used for intracranial SFT, but its use in spinal SFT is sporadic and no conclusion can be drawn. Conventional chemotherapy gives a poor clinical benefit, and anti-angiogenic treatments are the most promising option [ 35 , 36 , 37 ], used in one patient in our series, as a third-line option.…”

Section: Discussionmentioning

confidence: 99%

Spinal Solitary Fibrous Tumors: An Original Multicenter Series and Systematic Review of Presentation, Management, and Prognosis

Apra

Arbi

Montero

et al. 2022

Cancers

Self Cite

View full text Add to dashboard Cite

All solitary fibrous tumors (SFT), now histologically diagnosed by a positive nuclear STAT6 immunostaining, represent less than 2% of soft tissue sarcomas, with spinal SFT constituting a maximum of 2% of them, making these tumors extremely rare. We provide an up-to-date overview of their diagnosis, treatment, and prognosis. We included 10 primary STAT6-positive SFT from our retrospective cohort and 31 from a systematic review. Spinal pain was the most common symptom, in 69% of patients, and the only one in 34%, followed by spinal cord compression in 41%, radicular compression, including pain or deficit, in 36%, and urinary dysfunction specifically in 18%. Preoperative diagnosis was never obtained. Gross total resection was achieved in 71%, in the absence of spinal cord invasion or excessive bleeding. Histologically, they were 35% grade I, 25% grade II, and 40% grade III. Recurrence was observed in 43% after a mean 5.8 years (1 to 25). No significant risk factor was identified, but adjuvant radiotherapy improved the recurrence-free survival after subtotal resection. In conclusion, spinal SFT must be treated by neurosurgeons as part of a multidisciplinary team. Owing to their close relationship with the spinal cord, radiotherapy should be considered when gross total resection cannot be achieved, to lower the risk of recurrence.

show abstract

“…Since 2012, pazopanib hydrochloride, a broad-spectrum tyrosine kinase inhibitor, has been approved for the treatment of soft tissue sarcoma in Japan, and its effectiveness has been reported in several papers ( 36 , 37 ). In this study, three patients (Case VII, XV and XI) had been treated with pazopanib hydrochloride, however, Case VII patient passed away within six months following its administration.…”

Section: Discussionmentioning

confidence: 99%

Fascin‑1 is associated with recurrence in solitary fibrous tumor/hemangiopericytoma

et al. 2021

View full text Add to dashboard Cite

Fascin-1, an actin-bundling protein, is associated with poor prognosis in patients with various types of human carcinoma. However, research is limited on the role of fascin-1 in sarcoma. Solitary fibrous tumor (SFT) and hemangiopericytoma (HPC) are rare sarcomas derived from the mesenchyme. Although the prognosis of SFT/HPC is generally favorable, fatalities are possible with repeated recurrence and distant metastasis. The current study included a total of 20 Japanese patients, who were diagnosed with SFT/HPC and underwent surgery at Kochi University Hospital from January 2000 to December 2019. The statistical relationship between recurrence and the following variables were examined: Sex, age of onset, tumor origin, tumor size, necrosis, mitosis ≥1/10 high power field (HPF; magnification, x400), Ki-67 >5% and Fascin-1. A significant association was determined between recurrence and necrosis, mitosis ≥1/10 HPF (magnification, x400), Ki-67 >5%, and Fascin-1 ≥'strongly positive' (P<0.05). The results demonstrated that Fascin-1 immunostaining may be a highly effective and useful evaluation factor for predicting poor prognosis in patients with SFT/HPC, a fatal sarcoma of humans.

show abstract

Pazopanib efficacy in recurrent central nervous system hemangiopericytomas

Abstract: Two consecutive patients with untreatable central nervous system SFT/H showed a spectacular partial response to Pazopanib, an unprecedented result in SFT/H. This result could be explained by differences in expression profiles and calls for a confirmation in a larger cohort of patients.

Cited by 12 publications

References 17 publications

Molecular description of meningeal solitary fibrous tumors/hemangiopericytomas compared to meningiomas: two completely separate entities

Molecular description of meningeal solitary fibrous tumors/hemangiopericytomas compared to meningiomas: two completely separate entities

Spinal Solitary Fibrous Tumors: An Original Multicenter Series and Systematic Review of Presentation, Management, and Prognosis

Fascin‑1 is associated with recurrence in solitary fibrous tumor/hemangiopericytoma

Contact Info

Product

Resources

About