Materiały źródłowe

• #1 Novel Therapeutic Options for Solitary Fibrous Tumor: Antiangiogenic Therapy and Beyond

  https://www.mdpi.com/2072-6694/14/4/1064

  Patients with SFTs should be managed within sarcoma reference centers, by a dedicated multidisciplinary team with a pathologist, radiologist, surgical oncologist, radiation oncologist, and medical oncologist who are familiar with the nuances of this disease. Each case has to be discussed in a specialized multidisciplinary tumor board (MTB) to determine the best individualized therapeutic strategy. […] Complete en bloc surgical resection with negative margins (R0) is the gold-standard treatment for localized disease. The 10 year overall survival (OS) in patients with SFTs resected with negative margins ranges from 54% to 89%. […] In patients with localized, resectable SFTs, there is no evidence supporting the use of systemic therapies in the (neo)adjuvant setting. […] In cases of synchronous and/or unresectable lung metastases and in cases of extrapulmonary metastatic disease, patients are candidates for systemic treatment, even though a standard medical approach is currently not established.

• #2 Solitary Fibrous Tumors – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK585038/

  Solitary fibrous tumors (SFTs) represent a rare type of mesenchymal neoplasm that can arise in various locations throughout the body, although most commonly occur in the thoracic cavity. […] Treatment typically involves surgical resection, although adjuvant therapies such as radiation and chemotherapy may be considered in cases of unresectable or metastatic disease. […] Due to their rarity and lack of randomized control trials, there is no global consensus on treating SFTs. At present, the most effective therapeutic modality for SFTs is surgical resection. The role of other treatment modalities in the management of SFTs is unclear. Some reports in the literature have demonstrated radiotherapy’s effectiveness in controlling SFTs. Chemotherapeutic drugs and novel targeted drugs seem to exert some activity on SFTs. However, a consensus has not been achieved concerning the effectiveness of radiotherapy and chemotherapy against SFTs. Close multidisciplinary collaboration is essential for optimal management and outcomes in patients with SFTs.

• #3 Novel Therapeutic Options for Solitary Fibrous Tumor: Antiangiogenic Therapy and Beyond

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8870479/

  In case of advanced disease, antiangiogenic therapy might be viewed as the best therapeutic option. […] Total en bloc resection is the standard treatment of patients with a localized SFT; in case of advanced disease, the clinical efficacy of conventional chemotherapy remains poor. […] Overall, antiangiogenic therapies showed a significant activity toward SFT in the advanced/metastatic setting. […] While the therapeutic potential of immunotherapy remains elusive, the use of antiangiogenics as first-line treatment should be considered. […] Complete en bloc surgical resection with negative margins (R0) is the gold-standard treatment for localized disease. […] In summary, observation is recommended in patients with negative margins (R0) without high-risk histologic features. […] In the case of intermediate- to high-risk SFT with positive margins (R1/R2), re-resection should be discussed for fit patients if complete resection can be achieved with minimal morbidity.

• #4 Solitary Fibrous Tumors – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK585038/

  Surgical resection, ideally achieving an R0 resection, is the primary treatment modality for localized SFTs and in cases of oligometastatic disease. […] The role of adjuvant radiation therapy is local control, especially in tumors at high risk for recurrence. […] Chemotherapy has traditionally been utilized in managing advanced or metastatic SFTs, although prospective evidence regarding its efficacy is limited. […] Antiangiogenic agents have emerged as promising therapeutic options for non-DD-SFT, showing superior activity to traditional chemotherapy.

• #5 Novel Therapeutic Options for Solitary Fibrous Tumor: Antiangiogenic Therapy and Beyond

  https://www.mdpi.com/2072-6694/14/4/1064

  Patients with SFTs should be managed within sarcoma reference centers, by a dedicated multidisciplinary team with a pathologist, radiologist, surgical oncologist, radiation oncologist, and medical oncologist who are familiar with the nuances of this disease. Each case has to be discussed in a specialized multidisciplinary tumor board (MTB) to determine the best individualized therapeutic strategy. […] Complete en bloc surgical resection with negative margins (R0) is the gold-standard treatment for localized disease. The 10 year overall survival (OS) in patients with SFTs resected with negative margins ranges from 54% to 89%. […] In patients with localized, resectable SFTs, there is no evidence supporting the use of systemic therapies in the (neo)adjuvant setting. […] In cases of synchronous and/or unresectable lung metastases and in cases of extrapulmonary metastatic disease, patients are candidates for systemic treatment, even though a standard medical approach is currently not established.

• #6 Malignant solitary fibrous tumor of the pleura | Journal of Cardiothoracic Surgery | Full Text

  https://cardiothoracicsurgery.biomedcentral.com/articles/10.1186/s13019-022-01842-6

  Solitary fibrous tumors of the pleura are rare diseases of the thoracic cavity. They frequently grow unnoticed until they exert compressive effects on adjacent organs. Treatment of solitary fibrous tumors of the pleura is surgical resection. Post-operative surveillance is recommended to identify early recurrent disease. […] Surgical resection is the treatment of choice followed by close post-operative surveillance. […] Complete en bloc surgical resection is the primary treatment for SFTP. For small tumors (5 cm), thoracoscopic approaches are used for resection, whereas larger tumors often utilize thoracotomy with wedge resection, pneumonectomy, segmentectomy, or lobectomy. Further resections of the chest wall or pericardium may be needed depending on the adhesion borders of the tumor. Post-operative treatment often includes a combination of temozolomide and bevacizumab which are found to have a high disease control when given together. This was given to our patient. […] Careful post-operative monitoring is advised after surgery due to the possibility of recurrence.

• #7 Malignant solitary fibrous tumor of the pleura | Journal of Cardiothoracic Surgery | Full Text

  https://cardiothoracicsurgery.biomedcentral.com/articles/10.1186/s13019-022-01842-6

  Solitary fibrous tumors of the pleura are rare diseases of the thoracic cavity. They frequently grow unnoticed until they exert compressive effects on adjacent organs. Treatment of solitary fibrous tumors of the pleura is surgical resection. Post-operative surveillance is recommended to identify early recurrent disease. […] Surgical resection is the treatment of choice followed by close post-operative surveillance. […] Complete en bloc surgical resection is the primary treatment for SFTP. For small tumors (5 cm), thoracoscopic approaches are used for resection, whereas larger tumors often utilize thoracotomy with wedge resection, pneumonectomy, segmentectomy, or lobectomy. Further resections of the chest wall or pericardium may be needed depending on the adhesion borders of the tumor. Post-operative treatment often includes a combination of temozolomide and bevacizumab which are found to have a high disease control when given together. This was given to our patient. […] Careful post-operative monitoring is advised after surgery due to the possibility of recurrence.

• #8 Solitary fibrous tumour | Sarcoma UK

  https://sarcoma.org.uk/about-sarcoma/what-is-sarcoma/types-of-sarcoma/solitary-fibrous-tumour/

  Surgery is the first treatment method for SFT. The surgeon will remove the tumour and will aim to take out an area of normal tissue too also called taking a margin. This is to make sure the surgeon removes all the cancer. […] If SFT is affecting the arms and legs, the surgeons will do limb-sparing surgery. This is so the limb can continue to work well. […] If the cancer has spread throughout the limb, surgeons may have to perform a partial or full amputation. This is very rare. […] This treatment uses high-energy radiation to destroy cancer cells. […] You may have radiotherapy either before or after surgery. Before surgery, radiotherapy aims to shrink the tumour, making it easier to remove. After surgery, radiotherapy aims to kill any remaining cancer cells that surgery didn’t remove.

• #9 Malignant solitary fibrous tumor of the pleura | Journal of Cardiothoracic Surgery | Full Text

  https://cardiothoracicsurgery.biomedcentral.com/articles/10.1186/s13019-022-01842-6

  Solitary fibrous tumors of the pleura are rare diseases of the thoracic cavity. They frequently grow unnoticed until they exert compressive effects on adjacent organs. Treatment of solitary fibrous tumors of the pleura is surgical resection. Post-operative surveillance is recommended to identify early recurrent disease. […] Surgical resection is the treatment of choice followed by close post-operative surveillance. […] Complete en bloc surgical resection is the primary treatment for SFTP. For small tumors (5 cm), thoracoscopic approaches are used for resection, whereas larger tumors often utilize thoracotomy with wedge resection, pneumonectomy, segmentectomy, or lobectomy. Further resections of the chest wall or pericardium may be needed depending on the adhesion borders of the tumor. Post-operative treatment often includes a combination of temozolomide and bevacizumab which are found to have a high disease control when given together. This was given to our patient. […] Careful post-operative monitoring is advised after surgery due to the possibility of recurrence.

• #10 Novel Therapeutic Options for Solitary Fibrous Tumor: Antiangiogenic Therapy and Beyond

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8870479/

  In case of advanced disease, antiangiogenic therapy might be viewed as the best therapeutic option. […] Total en bloc resection is the standard treatment of patients with a localized SFT; in case of advanced disease, the clinical efficacy of conventional chemotherapy remains poor. […] Overall, antiangiogenic therapies showed a significant activity toward SFT in the advanced/metastatic setting. […] While the therapeutic potential of immunotherapy remains elusive, the use of antiangiogenics as first-line treatment should be considered. […] Complete en bloc surgical resection with negative margins (R0) is the gold-standard treatment for localized disease. […] In summary, observation is recommended in patients with negative margins (R0) without high-risk histologic features. […] In the case of intermediate- to high-risk SFT with positive margins (R1/R2), re-resection should be discussed for fit patients if complete resection can be achieved with minimal morbidity.

• #11 Surgical treatment of primary solitary fibrous tumors involving the pelvic ring | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0207581

  Surgical treatment is the preferred treatment method for this disease. […] To the best of our knowledge, this study includes the largest number of cases of primary solitary fibrous tumors involving the pelvic ring to date. […] Most studies in the literature suggest that wide resection and en bloc resection are necessary for improving the postoperative local control rate and the overall survival of solitary fibrous tumors. […] However, this study shows that piecemeal resection of the total tumor can also achieve good local control. […] There was no significant difference in the progression-free survival rates of the piecemeal resection group compared with the en bloc resection group. […] If en bloc resection is not feasible, piecemeal resection can also achieve acceptable local control, particularly for patients with sacral tumors, as this approach can result in even better postoperative function than that achieved by other approaches.

• #12 Solitary fibrous tumor – Overview – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/solitary-fibrous-tumors/cdc-20395823

  Solitary fibrous tumors are growths of cells that can form in almost any part of the body. […] Treatment for solitary fibrous tumor often involves surgery to remove the tumor. Sometimes the tumor comes back after surgery or spreads to other parts of the body. […] Most often, surgery is the only treatment needed for a solitary fibrous tumor. Surgeons remove the tumor and a small amount of healthy tissue around it. […] Other treatments might be used after surgery to lower the risk that the tumor will come back. These other treatments might include radiation therapy or chemotherapy. […] Radiation therapy uses powerful energy beams to kill tumor cells. […] Chemotherapy uses strong medicines to kill tumor cells. For a solitary fibrous tumor, chemotherapy might be used if the tumor has spread or can’t be removed with surgery. […] Targeted therapy uses medicines that attack specific chemicals in the tumor cells. By blocking these chemicals, targeted treatments can kill tumor cells. Targeted therapy might be used if a solitary fibrous tumor has spread to other parts of the body.

• #13 Solitary fibrous tumor | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/solitary-fibrous-tumor?content_id=CON-20198292

  This rare type of tumor most often occurs near the lungs. Surgery is usually the treatment. […] Treatment for solitary fibrous tumors often involves surgery. Other treatments may include radiation therapy, chemotherapy and targeted therapy. […] Most often, surgery is the only treatment needed for a solitary fibrous tumor. Surgeons remove the tumor and a small amount of healthy tissue around it. The type of operation used to remove a solitary fibrous tumor depends on where the tumor is in the body. […] Other treatments might be used after surgery to lower the risk that the tumor will come back. These other treatments might include radiation therapy or chemotherapy. […] Radiation therapy might be used before or after surgery to lower the risk that the solitary fibrous tumor will come back. Sometimes radiation therapy is used instead of surgery. This might happen if the tumor is in a location that is not safe to do surgery. […] Chemotherapy might be used if the tumor has spread or can’t be removed with surgery. […] Targeted therapy might be used if a solitary fibrous tumor has spread to other parts of the body.

• #14 Solitary fibrous tumor – Davanzo – Translational Gastroenterology and Hepatology

  https://tgh.amegroups.org/article/view/4708/html

  Surgical management has been the mainstay treatment for SFTs. […] Given the variable location of the primary tumor, surgical planning and intervention is highly variable based upon location and involved structures. Surgical management of SFTs is similar to most soft tissue sarcomas with a goal of wide resection margins and preservation of any critical surrounding organs or other structures. Obtaining adequate negative margins has been shown to decrease the rate local disease recurrence and improve survival. […] The use of radiation therapy in treatment of SFTs is unfortunately limited by lack of data given the rarity of this tumor. […] Although the role of adjuvant radiation for non-intracranial tumors is still unclear, small case series have suggested potential encouraging results. […] The relatively low incidence of SFTs has made it difficult to identify the most effective chemotherapy for advanced SFT. […] As effectiveness of these chemotherapies is still relatively poor, additional clinical studies are needed to better elucidate the molecular pathways involved with SFT in order to treat it more effectively.

• #15 Solitary fibrous tumor | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/solitary-fibrous-tumour?lang=us

  Solitary fibrous tumors are a rare neoplasm of mesenchymal origin that comprise less than 2% of all soft tissue tumors ranging from indolent tumors to more aggressive masses. […] Lower risk solitary fibrous tumors tend to be smaller (10 cm) in size and occur in younger individuals. They typically have a favorable outcome with surgical resection alone. […] In contrast, high risk tumors tend to be larger at the time of diagnosis (10 cm), more commonly encountered in older individuals and have the expected histological features (increased cellularity, increased mitotic activity, nuclear pleomorphism, etc.) as well as necrosis and local infiltration. These have a poorer prognosis, especially if incompletely resected. […] Optimal adjuvant therapy for this group is unknown, and close-interval follow-up is advised because there is an increased incidence of local recurrence.

• #16 Surgical and radiation treatment of a paravertebral malignant solitary fibrous tumor: a case report and literature review – Scher – AME Case Reports

  https://acr.amegroups.org/article/view/6600/html

  In the present case, the decision to offer post-operative radiation to the patient was largely based on the patients focally positive margins; taking into account the additional consideration of the large tumor size, there was sufficient support for adjuvant radiation to a post-operative dose of 60 Gy in 30 fractions. […] Post-operative radiation treatment may improve rates of local control especially in the setting of concern for gross residual tumor or positive margins, subsequently preventing future morbidity and mortality.

• #17 The Role of Postoperative Radiotherapy in Intracranial Solitary Fibrous Tumor/Hemangiopericytoma: A Multi-institutional Retrospective Study (KROG 18-11)

  https://www.e-crt.org/journal/view.php?number=3237

  This study aimed to evaluate the role of postoperative radiotherapy (PORT) in intracranial solitary fibrous tumor/hemangiopericytoma (SFT/HPC). […] PORT was performed in 85 patients (64%). […] The multivariate analysis suggested that PORT significantly improved local control (LC) (p 0.001) and progression-free survival (PFS) (p 0.001). […] This multicenter study supports the role of PORT in disease control of intracranial SFT/HPC, irrespective of the surgical extent and grade. For LC, PORT should enclose the tumor bed with sufficient margin. […] Postoperative radiotherapy (PORT) delivered in patients undergoing STR for SFT/HPC has shown effective local control (LC). […] The present study revealed that WHO grade II is a prognostic factor that predicts lower extracranial metastasis even after adjusting for other confounders.

• #18 The Role of Postoperative Radiotherapy in Intracranial Solitary Fibrous Tumor/Hemangiopericytoma: A Multi-institutional Retrospective Study (KROG 18-11)

  https://www.e-crt.org/journal/view.php?number=3237

  The role of SRS in SFT/HPC has been studied in small cohort retrospective studies. […] The current study is the first to show that a sufficient margin of 1 cm for CTV may be required to encompass the microscopic residual tumor volume after surgery. […] In summary, we have demonstrated the disease control efficacy of PORT in SFT/HPC regardless of the extent of surgery and grade. These findings of this multicenter study, comprising a large number of patients compared to other retrospective studies, support the use of PORT in all SFT/HPC patients after surgery. For local control, PORT needs to enclose the residual tumor or surgical cavity with sufficient margin for the target volume.

• #19 Solitary fibrous tumor

  https://johnsonmemorial.org/jmh-health/disease-conditions/con-20198292

  Treatment for solitary fibrous tumor often involves: […] Most often, surgery is the only treatment needed for solitary fibrous tumors. Surgeons remove the tumor and a small amount of healthy tissue around it. The type of operation used to remove a solitary fibrous tumor depends on where the tumor is in the body. […] Other treatments might be used after surgery to lower the risk that the tumor will come back. These other treatments might include radiation or chemotherapy. […] Radiation therapy uses powerful energy beams to kill tumor cells. The energy can come from X-rays, protons or other sources. During radiation therapy, you lie on a table while a machine moves around you. The machine directs radiation to precise points on your body. […] Radiation might be used after surgery if all of the tumor can’t be removed. It might lower the risk that the tumor will come back after surgery. Sometimes radiation is used before surgery to shrink the tumor. This might make it more likely that the tumor is removed completely.

• #20

  https://link.springer.com/article/10.1007/s13691-012-0059-5

  Solitary fibrous tumor (SFT) is an uncommon neoplasm located in the pelvis. […] A hypervascular malignant SFT in the mesentery of the rectum showed a significant response to preoperative radiotherapy. […] The most effective therapeutic modality for SFTs is surgical resection. The role of the other treatment modalities, such as radiotherapy and chemotherapy, is presently unclear because of the rarity of SFTs. […] In the present case, the preoperative pelvic radiotherapy (total dose 50 Gy) successfully reduced the tumor size of a pelvic SFT. […] This case strongly supports the potential effectiveness of preoperative radiotherapy for the safe gross total removal of a large pelvic SFT, and it should be carefully considered as a therapeutic modality.

• #21 How would you approach therapy for a solitary fibrous tumor

  https://www.themednet.org/how-would-you-approach-therapy-for-a-solitary-fibrous-tumor-of-the-orbit-with-residual-disease-10-after-surgery

  The discussion about the possibility of complete resection, even if it involves orbital exenteration needs to happen since that is the only potentially curative treatment. […] If surgery is not an option or if it is deemed unacceptable by the patient, SFT is indeed radiosensitive.

• #22 Solitary fibrous tumor | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/solitary-fibrous-tumor

  Radiation therapy might be used before or after surgery to lower the risk that the solitary fibrous tumor will come back. Sometimes radiation therapy is used instead of surgery. This might happen if the tumor is in a location that is not safe to do surgery. […] Chemotherapy uses strong medicines to kill tumor cells. For a solitary fibrous tumor, chemotherapy might be used if the tumor has spread or can’t be removed with surgery. […] Targeted therapy uses medicines that attack specific chemicals in the tumor cells. By blocking these chemicals, targeted treatments can kill tumor cells. Targeted therapy might be used if a solitary fibrous tumor has spread to other parts of the body.

• #23 Solitary fibrous tumor – Overview – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/solitary-fibrous-tumors/cdc-20395823

  Solitary fibrous tumors are growths of cells that can form in almost any part of the body. […] Treatment for solitary fibrous tumor often involves surgery to remove the tumor. Sometimes the tumor comes back after surgery or spreads to other parts of the body. […] Most often, surgery is the only treatment needed for a solitary fibrous tumor. Surgeons remove the tumor and a small amount of healthy tissue around it. […] Other treatments might be used after surgery to lower the risk that the tumor will come back. These other treatments might include radiation therapy or chemotherapy. […] Radiation therapy uses powerful energy beams to kill tumor cells. […] Chemotherapy uses strong medicines to kill tumor cells. For a solitary fibrous tumor, chemotherapy might be used if the tumor has spread or can’t be removed with surgery. […] Targeted therapy uses medicines that attack specific chemicals in the tumor cells. By blocking these chemicals, targeted treatments can kill tumor cells. Targeted therapy might be used if a solitary fibrous tumor has spread to other parts of the body.

• #24 Solitary fibrous tumour | Sarcoma UK

  https://sarcoma.org.uk/about-sarcoma/what-is-sarcoma/types-of-sarcoma/solitary-fibrous-tumour/

  This treatment uses anti-cancer drugs to destroy cancer cells. Doctors may use it in patients who can’t have surgery, have a high risk of cancer returning, or if their cancer has spread (metastasis). […] In SFT, researchers are studying treatments called targeted therapies and immunotherapies. […] Targeted therapy is a treatment that stops cancer cells from growing and spreading. It targets specific characteristics within the cells. […] Immunotherapy is a treatment that helps your immune system kill cancer cells. […] Clinical trials have found that some targeted and immunotherapies work well for SFT. But, more research and trials are needed before these treatments are approved. […] After treatment, you will have regular follow-up appointments for several years. […] If the cancer reappears, you should have treatment as quickly as possible. Your treatment plan may include more surgery or radiotherapy, depending on what you need. […] If you have advanced cancer, your treatment may involve surgery, radiotherapy, or other therapies. Doctors will evaluate your treatment on a case-by-case basis.

• #25

  https://link.springer.com/article/10.1007/s10555-024-10204-8

  Solitary fibrous tumor (SFT) is a rare fibroblastic mesenchymal neoplasm. […] Traditional treatment strategies of SFT encompass surgical resection, radiation therapy, and chemotherapy, while emerging management regimes include antiangiogenic agents, immunotherapy, RNA-targeting technologies, and potential targeted drugs. […] The cornerstone for managing localized SFT is en bloc surgical resection with negative margins. […] For advanced or metastatic SFT, chemotherapy agents have been investigated. […] Emerging options such as antiangiogenic agents, immunotherapy, combination therapies, and RNA-targeting technologies are promising as innovative avenues for treating SFT. […] In cases of advanced or metastatic SFT, where surgery alone is insufficient, systemic treatment becomes a critical component. Treatment options encompass anthracycline-based therapy, Ifosfamide, and other chemotherapy agents typically utilized in soft tissue sarcoma treatment.

• #26 Novel Therapeutic Options for Solitary Fibrous Tumor: Antiangiogenic Therapy and Beyond

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8870479/

  Importantly, adjuvant CT should never be intended to rescue inadequate surgery. […] In summary, even if there is no formal gold standard, patients with metastatic SFTs are treated routinely in the same way as other STSs, using anthracycline as first-line therapy, while ifosfamide, dacarbazine, and trabectedin are options for second line and beyond. […] Prolonged disease control up to 30 months has been reported with antiangiogenic agents (sunitinib, sorafenib, pazopanib, temozolomide-bevacizumab) in case reports, retrospective studies, and phase I and phase II trials. […] Importantly, pazopanib can be a treatment option in the first-line setting for typical SFT. […] Overall, our review underlines the modest activity of standard chemotherapy in SFT but confirms that antiangiogenic agents have interesting activity and might be considered as the best therapeutic option in the advanced setting.

• #27 Novel Therapeutic Options for Solitary Fibrous Tumor: Antiangiogenic Therapy and Beyond

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8870479/

  Importantly, adjuvant CT should never be intended to rescue inadequate surgery. […] In summary, even if there is no formal gold standard, patients with metastatic SFTs are treated routinely in the same way as other STSs, using anthracycline as first-line therapy, while ifosfamide, dacarbazine, and trabectedin are options for second line and beyond. […] Prolonged disease control up to 30 months has been reported with antiangiogenic agents (sunitinib, sorafenib, pazopanib, temozolomide-bevacizumab) in case reports, retrospective studies, and phase I and phase II trials. […] Importantly, pazopanib can be a treatment option in the first-line setting for typical SFT. […] Overall, our review underlines the modest activity of standard chemotherapy in SFT but confirms that antiangiogenic agents have interesting activity and might be considered as the best therapeutic option in the advanced setting.

• #28 Malignant Hepatic Solitary Fibrous Tumor

  https://www.e-jlc.org/journal/view.php?number=432

  Hepatic solitary fibrous tumors (SFTs) are mostly benign and rare because of information regarding the clinical symptoms, treatment, and prognosis of their malignant forms is currently lacking. […] The patient refused palliative surgery and thus, received three cycles of chemotherapy (CTx) that included adriamycin (30 mg on days 1-3), ifosfamide (2,355 mg on days 2-4), and mesna (1,256 mg IV 0 hours, 3 hours, and 6 hours after ifosfamide). Adjunctive radiation therapy was not administered. […] Many authors believe that postoperative adjuvant CTx and radiotherapy are helpful in unresectability or malignancy cases. […] Currently, the combination of doxorubicin and ifosfamide is the standard systemic CTx regimen for many soft-tissue sarcoma subtypes. […] Additional investigations in a controlled, prospective trial on the use of CTx and its regimen to treat malignant SFTs are warranted. […] Further studies are required to evaluate the effectiveness of radiotherapy as an adjunctive therapy for malignant hepatic SFTs.

• #29 Novel Therapeutic Options for Solitary Fibrous Tumor: Antiangiogenic Therapy and Beyond

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8870479/

  Importantly, adjuvant CT should never be intended to rescue inadequate surgery. […] In summary, even if there is no formal gold standard, patients with metastatic SFTs are treated routinely in the same way as other STSs, using anthracycline as first-line therapy, while ifosfamide, dacarbazine, and trabectedin are options for second line and beyond. […] Prolonged disease control up to 30 months has been reported with antiangiogenic agents (sunitinib, sorafenib, pazopanib, temozolomide-bevacizumab) in case reports, retrospective studies, and phase I and phase II trials. […] Importantly, pazopanib can be a treatment option in the first-line setting for typical SFT. […] Overall, our review underlines the modest activity of standard chemotherapy in SFT but confirms that antiangiogenic agents have interesting activity and might be considered as the best therapeutic option in the advanced setting.

• #30 Novel Therapeutic Options for Solitary Fibrous Tumor: Antiangiogenic Therapy and Beyond

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8870479/

  Importantly, adjuvant CT should never be intended to rescue inadequate surgery. […] In summary, even if there is no formal gold standard, patients with metastatic SFTs are treated routinely in the same way as other STSs, using anthracycline as first-line therapy, while ifosfamide, dacarbazine, and trabectedin are options for second line and beyond. […] Prolonged disease control up to 30 months has been reported with antiangiogenic agents (sunitinib, sorafenib, pazopanib, temozolomide-bevacizumab) in case reports, retrospective studies, and phase I and phase II trials. […] Importantly, pazopanib can be a treatment option in the first-line setting for typical SFT. […] Overall, our review underlines the modest activity of standard chemotherapy in SFT but confirms that antiangiogenic agents have interesting activity and might be considered as the best therapeutic option in the advanced setting.

• #31 How do you approach treatment for solitary fibrous tumor

  https://www.themednet.org/how-do-you-approach-treatment-for-solitary-fibrous-tumor-with-a-multifocal-pleural-based-metastatic-recurrence-that-is-surgically-inoperable-in-a-patient-with-good-performance-status

  Standard sarcoma chemotherapy does NOT work unless SFT has dedifferentiated into a frank sarcoma. For SFT, VEGFR inhibition helps. Options tested are temozolomide/bevacizumab (Park, et al. Cancer 2011), sunitinib or pazopanib. […] In addition to the options put forth by @Shreyaskumar Patel, there is a retrospective describing activity of trabectedin for SFT: Efficacy of trabectedin in malignant solitary fibrous tumors: a retrospective analysis from the French Sarcoma Group. I have had some luck with trabectedin.

• #32

  https://journals.lww.com/md-journal/fulltext/2024/07190/total_management_of_hemangiopericytoma_solitary.57.aspx

  Solitary fibrous tumors can manifest at various anatomical sites, predominantly occurring at extrapleural sites with a peak incidence between 40 and 70 years. SFT necessitates long-term follow-up owing to its tumor characteristics. […] A 54-year-old woman had a T1 low-to-isobaric and T2 isobaric-to-hyperintense mass in the psoas muscle on magnetic resonance imaging, diagnosed as an SFT. Wide excision was performed, followed by postoperative radiotherapy and chemotherapy. […] The radiotherapy dose was 66 Gy; no complications occurred, and local recurrence was prevented. […] At the time of diagnosis, both weekly PTX and pazopanib were reportedly effective against hemangiopericytomas. […] The treatment course for SFTs at our hospital yielded favorable outcomes.

• #33 Malignant Hepatic Solitary Fibrous Tumor

  https://www.e-jlc.org/journal/view.php?number=432

  Hepatic solitary fibrous tumors (SFTs) are mostly benign and rare because of information regarding the clinical symptoms, treatment, and prognosis of their malignant forms is currently lacking. […] The patient refused palliative surgery and thus, received three cycles of chemotherapy (CTx) that included adriamycin (30 mg on days 1-3), ifosfamide (2,355 mg on days 2-4), and mesna (1,256 mg IV 0 hours, 3 hours, and 6 hours after ifosfamide). Adjunctive radiation therapy was not administered. […] Many authors believe that postoperative adjuvant CTx and radiotherapy are helpful in unresectability or malignancy cases. […] Currently, the combination of doxorubicin and ifosfamide is the standard systemic CTx regimen for many soft-tissue sarcoma subtypes. […] Additional investigations in a controlled, prospective trial on the use of CTx and its regimen to treat malignant SFTs are warranted. […] Further studies are required to evaluate the effectiveness of radiotherapy as an adjunctive therapy for malignant hepatic SFTs.

• #34 Novel Therapeutic Options for Solitary Fibrous Tumor: Antiangiogenic Therapy and Beyond

  https://www.mdpi.com/2072-6694/14/4/1064

  SFTs are highly vascularized tumors with high expression rates of proteins involved in angiogenic pathways such as platelet-derived growth factor receptor (PDGFR) and vascular endothelial growth factor receptor (VEGFR). […] Prolonged disease control up to 30 months has been reported with antiangiogenic agents (sunitinib, sorafenib, pazopanib, temozolomide–bevacizumab) in case reports, retrospective studies, and phase I and phase II trials. […] Importantly, pazopanib can be a treatment option in the first-line setting for typical SFT.

• #35 Solitary Fibrous Tumors – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK585038/

  Surgical resection, ideally achieving an R0 resection, is the primary treatment modality for localized SFTs and in cases of oligometastatic disease. […] The role of adjuvant radiation therapy is local control, especially in tumors at high risk for recurrence. […] Chemotherapy has traditionally been utilized in managing advanced or metastatic SFTs, although prospective evidence regarding its efficacy is limited. […] Antiangiogenic agents have emerged as promising therapeutic options for non-DD-SFT, showing superior activity to traditional chemotherapy.

• #36 Novel Therapeutic Options for Solitary Fibrous Tumor: Antiangiogenic Therapy and Beyond

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8870479/

  Importantly, adjuvant CT should never be intended to rescue inadequate surgery. […] In summary, even if there is no formal gold standard, patients with metastatic SFTs are treated routinely in the same way as other STSs, using anthracycline as first-line therapy, while ifosfamide, dacarbazine, and trabectedin are options for second line and beyond. […] Prolonged disease control up to 30 months has been reported with antiangiogenic agents (sunitinib, sorafenib, pazopanib, temozolomide-bevacizumab) in case reports, retrospective studies, and phase I and phase II trials. […] Importantly, pazopanib can be a treatment option in the first-line setting for typical SFT. […] Overall, our review underlines the modest activity of standard chemotherapy in SFT but confirms that antiangiogenic agents have interesting activity and might be considered as the best therapeutic option in the advanced setting.

• #37 Novel Therapeutic Options for Solitary Fibrous Tumor: Antiangiogenic Therapy and Beyond

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8870479/

  Importantly, adjuvant CT should never be intended to rescue inadequate surgery. […] In summary, even if there is no formal gold standard, patients with metastatic SFTs are treated routinely in the same way as other STSs, using anthracycline as first-line therapy, while ifosfamide, dacarbazine, and trabectedin are options for second line and beyond. […] Prolonged disease control up to 30 months has been reported with antiangiogenic agents (sunitinib, sorafenib, pazopanib, temozolomide-bevacizumab) in case reports, retrospective studies, and phase I and phase II trials. […] Importantly, pazopanib can be a treatment option in the first-line setting for typical SFT. […] Overall, our review underlines the modest activity of standard chemotherapy in SFT but confirms that antiangiogenic agents have interesting activity and might be considered as the best therapeutic option in the advanced setting.

• #38 Treating Typical Solitary Fibrous Tumor With a Tyrosine Kinase Inhibitor – The ASCO Post

  https://ascopost.com/news/february-2020/treating-typical-solitary-fibrous-tumor-with-a-tyrosine-kinase-inhibitor/

  In a European phase II trial reported in The Lancet Oncology, Martin-Broto et al found that pazopanib showed activity in patients with typical solitary fibrous tumors. […] The manageable toxicity and activity shown by pazopanib in this cohort suggest that this drug could be considered as first-line treatment for advanced typical solitary fibrous tumor. […] The investigators concluded, To our knowledge, this is the first prospective trial of pazopanib for advanced typical solitary fibrous tumor. The manageable toxicity and activity shown by pazopanib in this cohort suggest that this drug could be considered as first-line treatment for advanced typical solitary fibrous tumor.

• #39 Novel Therapeutic Options for Solitary Fibrous Tumor: Antiangiogenic Therapy and Beyond

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8870479/

  Importantly, adjuvant CT should never be intended to rescue inadequate surgery. […] In summary, even if there is no formal gold standard, patients with metastatic SFTs are treated routinely in the same way as other STSs, using anthracycline as first-line therapy, while ifosfamide, dacarbazine, and trabectedin are options for second line and beyond. […] Prolonged disease control up to 30 months has been reported with antiangiogenic agents (sunitinib, sorafenib, pazopanib, temozolomide-bevacizumab) in case reports, retrospective studies, and phase I and phase II trials. […] Importantly, pazopanib can be a treatment option in the first-line setting for typical SFT. […] Overall, our review underlines the modest activity of standard chemotherapy in SFT but confirms that antiangiogenic agents have interesting activity and might be considered as the best therapeutic option in the advanced setting.

• #40 Solitary fibrous tumor – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube

  https://www.augustahealth.com/disease/solitary-fibrous-tumor/

  In most cases surgery is the only treatment necessary. Surgeons remove the tumor and a small margin of healthy tissue that surrounds it. The type of operation used to remove a solitary fibrous tumor depends on where the tumor occurs. […] If the entire tumor can’t be removed, your doctor might suggest radiation therapy. Sometimes, radiation therapy is used before surgery to shrink the tumor. This increases the chances that the entire tumor can be removed. Chemotherapy is another treatment option, particularly if the tumor has spread to other parts of your body. […] Certain drugs target the vascular endothelial growth factor (VEGF) and other tyrosine kinase signaling pathways. These pathways disrupt the blood supply to the tumor, and have recently begun to be used to treat advanced solitary fibrous tumors to slow their progression. Examples of these drugs include bevacizumab (Avastin), sunitinib (Sutent), pazopanib (Votrient) and sorafenib (Nexavar).

• #41 Solitary fibrous tumor – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube

  https://www.augustahealth.com/disease/solitary-fibrous-tumor/

  In most cases surgery is the only treatment necessary. Surgeons remove the tumor and a small margin of healthy tissue that surrounds it. The type of operation used to remove a solitary fibrous tumor depends on where the tumor occurs. […] If the entire tumor can’t be removed, your doctor might suggest radiation therapy. Sometimes, radiation therapy is used before surgery to shrink the tumor. This increases the chances that the entire tumor can be removed. Chemotherapy is another treatment option, particularly if the tumor has spread to other parts of your body. […] Certain drugs target the vascular endothelial growth factor (VEGF) and other tyrosine kinase signaling pathways. These pathways disrupt the blood supply to the tumor, and have recently begun to be used to treat advanced solitary fibrous tumors to slow their progression. Examples of these drugs include bevacizumab (Avastin), sunitinib (Sutent), pazopanib (Votrient) and sorafenib (Nexavar).

• #42 Malignant solitary fibrous tumor of the pleura | Journal of Cardiothoracic Surgery | Full Text

  https://cardiothoracicsurgery.biomedcentral.com/articles/10.1186/s13019-022-01842-6

  Solitary fibrous tumors of the pleura are rare diseases of the thoracic cavity. They frequently grow unnoticed until they exert compressive effects on adjacent organs. Treatment of solitary fibrous tumors of the pleura is surgical resection. Post-operative surveillance is recommended to identify early recurrent disease. […] Surgical resection is the treatment of choice followed by close post-operative surveillance. […] Complete en bloc surgical resection is the primary treatment for SFTP. For small tumors (5 cm), thoracoscopic approaches are used for resection, whereas larger tumors often utilize thoracotomy with wedge resection, pneumonectomy, segmentectomy, or lobectomy. Further resections of the chest wall or pericardium may be needed depending on the adhesion borders of the tumor. Post-operative treatment often includes a combination of temozolomide and bevacizumab which are found to have a high disease control when given together. This was given to our patient. […] Careful post-operative monitoring is advised after surgery due to the possibility of recurrence.

• #43 Solitary fibrous tumor – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube

  https://www.augustahealth.com/disease/solitary-fibrous-tumor/

  In most cases surgery is the only treatment necessary. Surgeons remove the tumor and a small margin of healthy tissue that surrounds it. The type of operation used to remove a solitary fibrous tumor depends on where the tumor occurs. […] If the entire tumor can’t be removed, your doctor might suggest radiation therapy. Sometimes, radiation therapy is used before surgery to shrink the tumor. This increases the chances that the entire tumor can be removed. Chemotherapy is another treatment option, particularly if the tumor has spread to other parts of your body. […] Certain drugs target the vascular endothelial growth factor (VEGF) and other tyrosine kinase signaling pathways. These pathways disrupt the blood supply to the tumor, and have recently begun to be used to treat advanced solitary fibrous tumors to slow their progression. Examples of these drugs include bevacizumab (Avastin), sunitinib (Sutent), pazopanib (Votrient) and sorafenib (Nexavar).

• #44 Malignant solitary fibrous tumor in the central nervous system treated with surgery, radiotherapy and anlotinib: A case report

  https://www.wjgnet.com/2307-8960/full/v10/i2/631.htm

  Malignant solitary fibrous tumor in the central nervous system treated with surgery, radiotherapy and anlotinib: A case report. […] There is no established standardized treatment regimen for malignant intracranial SFTs. […] The main treatments are surgical resection and postoperative radiotherapy. […] The patient received 60 Gy and 30 fractions of intensity modulated radiotherapy. […] Considering her pathological diagnosis, FGFR4 and TP53 mutation and progression of the disease, the oncologist at Beijing Cancer Hospital advised the patient to undergo anlotinib treatment. […] Anlotinib, a newly designed oral small-molecule receptor tyrosine kinase inhibitor, was developed independently by Chia-Tai Tianqing Pharmaceutical Co., Ltd. in China. […] This is the first report in the world of a patient with malignant intracranial SFT treated with surgery, radiotherapy and anlotinib monotherapy. […] Based on preliminary data, we speculated that FGFR 4 and TP53 mutations might be beneficial in the treatment of malignant intracranial SFT with anlotinib.

• #45 Novel Therapeutic Options for Solitary Fibrous Tumor: Antiangiogenic Therapy and Beyond

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8870479/

  Importantly, adjuvant CT should never be intended to rescue inadequate surgery. […] In summary, even if there is no formal gold standard, patients with metastatic SFTs are treated routinely in the same way as other STSs, using anthracycline as first-line therapy, while ifosfamide, dacarbazine, and trabectedin are options for second line and beyond. […] Prolonged disease control up to 30 months has been reported with antiangiogenic agents (sunitinib, sorafenib, pazopanib, temozolomide-bevacizumab) in case reports, retrospective studies, and phase I and phase II trials. […] Importantly, pazopanib can be a treatment option in the first-line setting for typical SFT. […] Overall, our review underlines the modest activity of standard chemotherapy in SFT but confirms that antiangiogenic agents have interesting activity and might be considered as the best therapeutic option in the advanced setting.

• #46 Novel Therapeutic Options for Solitary Fibrous Tumor: Antiangiogenic Therapy and Beyond

  https://www.mdpi.com/2072-6694/14/4/1064

  SFTs are highly vascularized tumors with high expression rates of proteins involved in angiogenic pathways such as platelet-derived growth factor receptor (PDGFR) and vascular endothelial growth factor receptor (VEGFR). […] Prolonged disease control up to 30 months has been reported with antiangiogenic agents (sunitinib, sorafenib, pazopanib, temozolomide–bevacizumab) in case reports, retrospective studies, and phase I and phase II trials. […] Importantly, pazopanib can be a treatment option in the first-line setting for typical SFT.

• #47

  https://link.springer.com/article/10.1007/s10555-024-10204-8

  It is worth noting that an evolving area in the treatment of SFT is the utilization of antiangiogenic agents. […] In addition, immunotherapy is increasingly recognized as a potential avenue for SFT treatment. […] A preclinical report has highlighted the potential of RNA-targeting technologies as a therapy for SFT. […] Currently, SFT lacks a specifically tailored treatment, but the NAB2-STAT6 and EGR1 hold as promised targets.

• #48 Comprehensive analysis reveals potential therapeutic targets and an integrated risk stratification model for solitary fibrous tumors | Nature Communications

  https://www.nature.com/articles/s41467-023-43249-4

  Our findings hold promise for advancing therapeutic strategies and refining risk prediction in SFTs. […] In this study, we found that the tumor-infiltrating immune cells in SFTs were predominantly macrophages, and high infiltration of macrophages was significantly associated with short PFS of the patients. Furthermore, we observed that 24.4% (32/131) of SFTs exhibited high PD-L1 expression in either tumor cells or in immune cells. […] The PD-1/PD-L1 axis inhibition alone is not enough for SFTs treatment, and double inhibition of angiogenesis and PD-1/PD-L1 axis was demonstrated as an effective treatment strategy, supporting combination strategies promoting inflamed microenvironment resulted in a higher efficacy in SFTs. […] In conclusion, our study has identified the mutation of IDH1 p.R132S and PD-L1 expression as potential therapeutic targets for SFTs, offering the possibility of more precise treatment options through combination targeted therapy or immunotherapy based on biomarkers screening.

• #49 Comprehensive analysis reveals potential therapeutic targets and an integrated risk stratification model for solitary fibrous tumors | Nature Communications

  https://www.nature.com/articles/s41467-023-43249-4

  Our findings hold promise for advancing therapeutic strategies and refining risk prediction in SFTs. […] In this study, we found that the tumor-infiltrating immune cells in SFTs were predominantly macrophages, and high infiltration of macrophages was significantly associated with short PFS of the patients. Furthermore, we observed that 24.4% (32/131) of SFTs exhibited high PD-L1 expression in either tumor cells or in immune cells. […] The PD-1/PD-L1 axis inhibition alone is not enough for SFTs treatment, and double inhibition of angiogenesis and PD-1/PD-L1 axis was demonstrated as an effective treatment strategy, supporting combination strategies promoting inflamed microenvironment resulted in a higher efficacy in SFTs. […] In conclusion, our study has identified the mutation of IDH1 p.R132S and PD-L1 expression as potential therapeutic targets for SFTs, offering the possibility of more precise treatment options through combination targeted therapy or immunotherapy based on biomarkers screening.

• #50 Comprehensive analysis reveals potential therapeutic targets and an integrated risk stratification model for solitary fibrous tumors | Nature Communications

  https://www.nature.com/articles/s41467-023-43249-4

  Our findings hold promise for advancing therapeutic strategies and refining risk prediction in SFTs. […] In this study, we found that the tumor-infiltrating immune cells in SFTs were predominantly macrophages, and high infiltration of macrophages was significantly associated with short PFS of the patients. Furthermore, we observed that 24.4% (32/131) of SFTs exhibited high PD-L1 expression in either tumor cells or in immune cells. […] The PD-1/PD-L1 axis inhibition alone is not enough for SFTs treatment, and double inhibition of angiogenesis and PD-1/PD-L1 axis was demonstrated as an effective treatment strategy, supporting combination strategies promoting inflamed microenvironment resulted in a higher efficacy in SFTs. […] In conclusion, our study has identified the mutation of IDH1 p.R132S and PD-L1 expression as potential therapeutic targets for SFTs, offering the possibility of more precise treatment options through combination targeted therapy or immunotherapy based on biomarkers screening.

• #51 Fibroblast Activation Protein α–Directed Imaging and Therapy of Solitary Fibrous Tumor | Journal of Nuclear Medicine

  https://jnm.snmjournals.org/content/65/2/252

  Fibroblast activation protein (FAP) is expressed at high levels in several types of tumors. Here, we report the expression pattern of FAP in solitary fibrous tumor (SFT) and its potential use as a radiotheranostic target. […] 90Y-FAPI-46 radioligand therapy was offered to eligible patients with progressive SFT. […] Eleven patients received a total of 34 cycles (median, 3 cycles [IQR, 2 cycles]) of 90Y-FAPI-46 radioligand therapy, which resulted in disease control in 9 patients (82%). […] FAP is highly expressed by SFT and may serve as a target for imaging and therapy. […] Therefore, better diagnosis and efficacious treatment are urgently needed. […] Here, we systematically explore FAP as a theranostic target in SFT. […] Our analysis showed that SFT samples demonstrated the highest median mRNA expression of FAP in the MASTER cohort.

• #52 Fibroblast Activation Protein α–Directed Imaging and Therapy of Solitary Fibrous Tumor | Journal of Nuclear Medicine

  https://jnm.snmjournals.org/content/65/2/252

  Fibroblast activation protein (FAP) is expressed at high levels in several types of tumors. Here, we report the expression pattern of FAP in solitary fibrous tumor (SFT) and its potential use as a radiotheranostic target. […] 90Y-FAPI-46 radioligand therapy was offered to eligible patients with progressive SFT. […] Eleven patients received a total of 34 cycles (median, 3 cycles [IQR, 2 cycles]) of 90Y-FAPI-46 radioligand therapy, which resulted in disease control in 9 patients (82%). […] FAP is highly expressed by SFT and may serve as a target for imaging and therapy. […] Therefore, better diagnosis and efficacious treatment are urgently needed. […] Here, we systematically explore FAP as a theranostic target in SFT. […] Our analysis showed that SFT samples demonstrated the highest median mRNA expression of FAP in the MASTER cohort.

• #53 Fibroblast Activation Protein α–Directed Imaging and Therapy of Solitary Fibrous Tumor | Journal of Nuclear Medicine

  https://jnm.snmjournals.org/content/65/2/252

  In line with the observed high FAP mRNA and protein expression, 18 of the 19 patients (95%) with SFT from the theranostic cohort showed high uptake of the FAP-directed radioligand 68Ga-FAPI-46 in tumor lesions. […] FAP-associated RLT is an emerging approach for targeted systemic therapy against solid tumors. […] We observed disease control in 9 patients (82%): partial response in 3 and stable disease in 6. […] These data strongly support future prospective evaluation of FAP-directed radioligand imaging and therapy in SFT and potentially other sarcomas.

• #54

  https://link.springer.com/article/10.1007/s10555-024-10204-8

  It is worth noting that an evolving area in the treatment of SFT is the utilization of antiangiogenic agents. […] In addition, immunotherapy is increasingly recognized as a potential avenue for SFT treatment. […] A preclinical report has highlighted the potential of RNA-targeting technologies as a therapy for SFT. […] Currently, SFT lacks a specifically tailored treatment, but the NAB2-STAT6 and EGR1 hold as promised targets.

• #55 Comprehensive analysis reveals potential therapeutic targets and an integrated risk stratification model for solitary fibrous tumors | Nature Communications

  https://www.nature.com/articles/s41467-023-43249-4

  Our findings hold promise for advancing therapeutic strategies and refining risk prediction in SFTs. […] In this study, we found that the tumor-infiltrating immune cells in SFTs were predominantly macrophages, and high infiltration of macrophages was significantly associated with short PFS of the patients. Furthermore, we observed that 24.4% (32/131) of SFTs exhibited high PD-L1 expression in either tumor cells or in immune cells. […] The PD-1/PD-L1 axis inhibition alone is not enough for SFTs treatment, and double inhibition of angiogenesis and PD-1/PD-L1 axis was demonstrated as an effective treatment strategy, supporting combination strategies promoting inflamed microenvironment resulted in a higher efficacy in SFTs. […] In conclusion, our study has identified the mutation of IDH1 p.R132S and PD-L1 expression as potential therapeutic targets for SFTs, offering the possibility of more precise treatment options through combination targeted therapy or immunotherapy based on biomarkers screening.

• #56

  https://link.springer.com/article/10.1007/s10555-024-10204-8

  It is worth noting that an evolving area in the treatment of SFT is the utilization of antiangiogenic agents. […] In addition, immunotherapy is increasingly recognized as a potential avenue for SFT treatment. […] A preclinical report has highlighted the potential of RNA-targeting technologies as a therapy for SFT. […] Currently, SFT lacks a specifically tailored treatment, but the NAB2-STAT6 and EGR1 hold as promised targets.

• #57 Novel Therapeutic Options for Solitary Fibrous Tumor: Antiangiogenic Therapy and Beyond

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8870479/

  In case of advanced disease, antiangiogenic therapy might be viewed as the best therapeutic option. […] Total en bloc resection is the standard treatment of patients with a localized SFT; in case of advanced disease, the clinical efficacy of conventional chemotherapy remains poor. […] Overall, antiangiogenic therapies showed a significant activity toward SFT in the advanced/metastatic setting. […] While the therapeutic potential of immunotherapy remains elusive, the use of antiangiogenics as first-line treatment should be considered. […] Complete en bloc surgical resection with negative margins (R0) is the gold-standard treatment for localized disease. […] In summary, observation is recommended in patients with negative margins (R0) without high-risk histologic features. […] In the case of intermediate- to high-risk SFT with positive margins (R1/R2), re-resection should be discussed for fit patients if complete resection can be achieved with minimal morbidity.

• #58 Novel Therapeutic Options for Solitary Fibrous Tumor: Antiangiogenic Therapy and Beyond

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8870479/

  In case of advanced disease, antiangiogenic therapy might be viewed as the best therapeutic option. […] Total en bloc resection is the standard treatment of patients with a localized SFT; in case of advanced disease, the clinical efficacy of conventional chemotherapy remains poor. […] Overall, antiangiogenic therapies showed a significant activity toward SFT in the advanced/metastatic setting. […] While the therapeutic potential of immunotherapy remains elusive, the use of antiangiogenics as first-line treatment should be considered. […] Complete en bloc surgical resection with negative margins (R0) is the gold-standard treatment for localized disease. […] In summary, observation is recommended in patients with negative margins (R0) without high-risk histologic features. […] In the case of intermediate- to high-risk SFT with positive margins (R1/R2), re-resection should be discussed for fit patients if complete resection can be achieved with minimal morbidity.

• #59 Novel Therapeutic Options for Solitary Fibrous Tumor: Antiangiogenic Therapy and Beyond

  https://www.mdpi.com/2072-6694/14/4/1064

  Patients with SFTs should be managed within sarcoma reference centers, by a dedicated multidisciplinary team with a pathologist, radiologist, surgical oncologist, radiation oncologist, and medical oncologist who are familiar with the nuances of this disease. Each case has to be discussed in a specialized multidisciplinary tumor board (MTB) to determine the best individualized therapeutic strategy. […] Complete en bloc surgical resection with negative margins (R0) is the gold-standard treatment for localized disease. The 10 year overall survival (OS) in patients with SFTs resected with negative margins ranges from 54% to 89%. […] In patients with localized, resectable SFTs, there is no evidence supporting the use of systemic therapies in the (neo)adjuvant setting. […] In cases of synchronous and/or unresectable lung metastases and in cases of extrapulmonary metastatic disease, patients are candidates for systemic treatment, even though a standard medical approach is currently not established.

• #60 Novel Therapeutic Options for Solitary Fibrous Tumor: Antiangiogenic Therapy and Beyond

  https://www.mdpi.com/2072-6694/14/4/1064

  Patients with SFTs should be managed within sarcoma reference centers, by a dedicated multidisciplinary team with a pathologist, radiologist, surgical oncologist, radiation oncologist, and medical oncologist who are familiar with the nuances of this disease. Each case has to be discussed in a specialized multidisciplinary tumor board (MTB) to determine the best individualized therapeutic strategy. […] Complete en bloc surgical resection with negative margins (R0) is the gold-standard treatment for localized disease. The 10 year overall survival (OS) in patients with SFTs resected with negative margins ranges from 54% to 89%. […] In patients with localized, resectable SFTs, there is no evidence supporting the use of systemic therapies in the (neo)adjuvant setting. […] In cases of synchronous and/or unresectable lung metastases and in cases of extrapulmonary metastatic disease, patients are candidates for systemic treatment, even though a standard medical approach is currently not established.

• #61 Treating Typical Solitary Fibrous Tumor With a Tyrosine Kinase Inhibitor – The ASCO Post

  https://ascopost.com/news/february-2020/treating-typical-solitary-fibrous-tumor-with-a-tyrosine-kinase-inhibitor/

  In a European phase II trial reported in The Lancet Oncology, Martin-Broto et al found that pazopanib showed activity in patients with typical solitary fibrous tumors. […] The manageable toxicity and activity shown by pazopanib in this cohort suggest that this drug could be considered as first-line treatment for advanced typical solitary fibrous tumor. […] The investigators concluded, To our knowledge, this is the first prospective trial of pazopanib for advanced typical solitary fibrous tumor. The manageable toxicity and activity shown by pazopanib in this cohort suggest that this drug could be considered as first-line treatment for advanced typical solitary fibrous tumor.

• #62 Novel Therapeutic Options for Solitary Fibrous Tumor: Antiangiogenic Therapy and Beyond

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8870479/

  Importantly, adjuvant CT should never be intended to rescue inadequate surgery. […] In summary, even if there is no formal gold standard, patients with metastatic SFTs are treated routinely in the same way as other STSs, using anthracycline as first-line therapy, while ifosfamide, dacarbazine, and trabectedin are options for second line and beyond. […] Prolonged disease control up to 30 months has been reported with antiangiogenic agents (sunitinib, sorafenib, pazopanib, temozolomide-bevacizumab) in case reports, retrospective studies, and phase I and phase II trials. […] Importantly, pazopanib can be a treatment option in the first-line setting for typical SFT. […] Overall, our review underlines the modest activity of standard chemotherapy in SFT but confirms that antiangiogenic agents have interesting activity and might be considered as the best therapeutic option in the advanced setting.

• #63 Solitary Fibrous Tumor Treatment Market Analysis Report by 2031

  https://www.transparencymarketresearch.com/solitary-fibrous-tumor-treatment-market.html

  The varying doses of external beam radiotherapy are being delivered to patients in order to improve clinical outcomes. […] It has been found that antiangiogenics are active and their sequential use from first line should be considered, except for dedifferentiated SFT for which chemotherapy is an optimum option. […] Immunohistochemistry is known to be one of the most sensitive and specific means of diagnosing SFT and is practical economical as well.

• #64 Solitary fibrous tumour | Sarcoma UK

  https://sarcoma.org.uk/about-sarcoma/what-is-sarcoma/types-of-sarcoma/solitary-fibrous-tumour/

  This treatment uses anti-cancer drugs to destroy cancer cells. Doctors may use it in patients who can’t have surgery, have a high risk of cancer returning, or if their cancer has spread (metastasis). […] In SFT, researchers are studying treatments called targeted therapies and immunotherapies. […] Targeted therapy is a treatment that stops cancer cells from growing and spreading. It targets specific characteristics within the cells. […] Immunotherapy is a treatment that helps your immune system kill cancer cells. […] Clinical trials have found that some targeted and immunotherapies work well for SFT. But, more research and trials are needed before these treatments are approved. […] After treatment, you will have regular follow-up appointments for several years. […] If the cancer reappears, you should have treatment as quickly as possible. Your treatment plan may include more surgery or radiotherapy, depending on what you need. […] If you have advanced cancer, your treatment may involve surgery, radiotherapy, or other therapies. Doctors will evaluate your treatment on a case-by-case basis.

• #65 Solitary Fibrous Tumor – EyeWiki

  https://eyewiki.org/Solitary_Fibrous_Tumor

  Complete surgical excision of the lesion or exenteration of infiltrating lesions are the recommended surgical treatments. […] Routine follow up can be recommended for patients in whom tumors are completely excised. Patients who have incomplete tumor removal should be followed more frequently and should have serial imaging to check for recurrence. […] Complications associated with surgical removal are typical for any orbital surgery including retrobulbar hemorrhage, diplopia, ptosis, strabismus, decreased vision, blindness, or incomplete tumor excision. Incomplete tumor excision may lead to recurrence and/or malignant degeneration. […] Prognosis is excellent for tumors that are completely excised. Of the cases described in the literature, about 75% of solitary fibrous tumors display a benign course; and 25% showing invasion to adjacent tissues, recurrence, or distant metastasis.

• #66 Solitary fibrous tumour – Overview of Information and Clinical Research

  https://clinicaltrials.eu/disease/solitary-fibrous-tumour/

  Chemotherapy is typically reserved for advanced or metastatic SFTs. While conventional chemotherapy has shown limited efficacy, it can help stabilize the disease in some patients. […] Antiangiogenic therapy has emerged as a promising treatment for SFTs, particularly in advanced or metastatic settings. […] Targeted therapy and immunotherapy are being explored as potential treatments for SFTs. […] After treatment, regular follow-up appointments are essential to monitor for any signs of recurrence. Even benign-appearing SFTs can recur locally or metastasize, necessitating long-term surveillance. […] The primary treatment for a solitary fibrous tumor is surgery, aiming for complete excision. Radiation therapy and chemotherapy may be used in conjunction with surgery or as alternatives in advanced cases.

• #67 Solitary fibrous tumour | Sarcoma UK

  https://sarcoma.org.uk/about-sarcoma/what-is-sarcoma/types-of-sarcoma/solitary-fibrous-tumour/

  This treatment uses anti-cancer drugs to destroy cancer cells. Doctors may use it in patients who can’t have surgery, have a high risk of cancer returning, or if their cancer has spread (metastasis). […] In SFT, researchers are studying treatments called targeted therapies and immunotherapies. […] Targeted therapy is a treatment that stops cancer cells from growing and spreading. It targets specific characteristics within the cells. […] Immunotherapy is a treatment that helps your immune system kill cancer cells. […] Clinical trials have found that some targeted and immunotherapies work well for SFT. But, more research and trials are needed before these treatments are approved. […] After treatment, you will have regular follow-up appointments for several years. […] If the cancer reappears, you should have treatment as quickly as possible. Your treatment plan may include more surgery or radiotherapy, depending on what you need. […] If you have advanced cancer, your treatment may involve surgery, radiotherapy, or other therapies. Doctors will evaluate your treatment on a case-by-case basis.

• #68 Novel Therapeutic Options for Solitary Fibrous Tumor: Antiangiogenic Therapy and Beyond

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8870479/

  In case of advanced disease, antiangiogenic therapy might be viewed as the best therapeutic option. […] Total en bloc resection is the standard treatment of patients with a localized SFT; in case of advanced disease, the clinical efficacy of conventional chemotherapy remains poor. […] Overall, antiangiogenic therapies showed a significant activity toward SFT in the advanced/metastatic setting. […] While the therapeutic potential of immunotherapy remains elusive, the use of antiangiogenics as first-line treatment should be considered. […] Complete en bloc surgical resection with negative margins (R0) is the gold-standard treatment for localized disease. […] In summary, observation is recommended in patients with negative margins (R0) without high-risk histologic features. […] In the case of intermediate- to high-risk SFT with positive margins (R1/R2), re-resection should be discussed for fit patients if complete resection can be achieved with minimal morbidity.

• #69 Novel Therapeutic Options for Solitary Fibrous Tumor: Antiangiogenic Therapy and Beyond

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8870479/

  Importantly, adjuvant CT should never be intended to rescue inadequate surgery. […] In summary, even if there is no formal gold standard, patients with metastatic SFTs are treated routinely in the same way as other STSs, using anthracycline as first-line therapy, while ifosfamide, dacarbazine, and trabectedin are options for second line and beyond. […] Prolonged disease control up to 30 months has been reported with antiangiogenic agents (sunitinib, sorafenib, pazopanib, temozolomide-bevacizumab) in case reports, retrospective studies, and phase I and phase II trials. […] Importantly, pazopanib can be a treatment option in the first-line setting for typical SFT. […] Overall, our review underlines the modest activity of standard chemotherapy in SFT but confirms that antiangiogenic agents have interesting activity and might be considered as the best therapeutic option in the advanced setting.

• #70 Novel Therapeutic Options for Solitary Fibrous Tumor: Antiangiogenic Therapy and Beyond

  https://www.mdpi.com/2072-6694/14/4/1064

  Patients with SFTs should be managed within sarcoma reference centers, by a dedicated multidisciplinary team with a pathologist, radiologist, surgical oncologist, radiation oncologist, and medical oncologist who are familiar with the nuances of this disease. Each case has to be discussed in a specialized multidisciplinary tumor board (MTB) to determine the best individualized therapeutic strategy. […] Complete en bloc surgical resection with negative margins (R0) is the gold-standard treatment for localized disease. The 10 year overall survival (OS) in patients with SFTs resected with negative margins ranges from 54% to 89%. […] In patients with localized, resectable SFTs, there is no evidence supporting the use of systemic therapies in the (neo)adjuvant setting. […] In cases of synchronous and/or unresectable lung metastases and in cases of extrapulmonary metastatic disease, patients are candidates for systemic treatment, even though a standard medical approach is currently not established.

• #71 Novel Therapeutic Options for Solitary Fibrous Tumor: Antiangiogenic Therapy and Beyond

  https://www.mdpi.com/2072-6694/14/4/1064

  Patients with SFTs should be managed within sarcoma reference centers, by a dedicated multidisciplinary team with a pathologist, radiologist, surgical oncologist, radiation oncologist, and medical oncologist who are familiar with the nuances of this disease. Each case has to be discussed in a specialized multidisciplinary tumor board (MTB) to determine the best individualized therapeutic strategy. […] Complete en bloc surgical resection with negative margins (R0) is the gold-standard treatment for localized disease. The 10 year overall survival (OS) in patients with SFTs resected with negative margins ranges from 54% to 89%. […] In patients with localized, resectable SFTs, there is no evidence supporting the use of systemic therapies in the (neo)adjuvant setting. […] In cases of synchronous and/or unresectable lung metastases and in cases of extrapulmonary metastatic disease, patients are candidates for systemic treatment, even though a standard medical approach is currently not established.

• #72 Solitary Fibrous Tumor Treatment Market Analysis Report by 2031

  https://www.transparencymarketresearch.com/solitary-fibrous-tumor-treatment-market.html

  Surgery remains the gold standard for increasing medical outcomes in the solitary fibrous tumor treatment market. […] Pazopanib holds promising potentials for advanced solitary fibrous tumor treatment. This multikinase inhibitor helps to address translocation-associated soft tissue sarcoma. […] Antiangiogenic treatment is found to be effective for unresectable disease. […] There is a need for translational work to understand the biology driving the differential behavior of SFT and identify more effective treatments for patients with metastatic disease. […] The solitary fibrous tumor treatment market is projected to expand at a modest CAGR of 4.5% during the forecast period. […] This has led to the adoption of the adjuvant radiation therapy to achieve local control in subtotally-resected pleural SFTs.

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