Solitary fibrous tumor represents a spectrum of mesenchymal tumors, encompassing tumors previously termed hemangiopericytoma, which are classified as having intermediate biological potential (rarely metastasizing) in the 2002 World Health Organization classification scheme. Few series have reported on clinicopathological predictors with outcome data and formal statistical analysis in a large series of primary tumors as a single unified entity. Institutional pathology records were reviewed to identify primary solitary fibrous tumor cases, and histological sections and clinical records reviewed for canonical prognostic indicators, including patient age, tumor size, mitotic index, tumor cellularity, nuclear pleomorphism, and tumor necrosis. Patients (n ¼ 103) with resected primary solitary fibrous tumor were identified (excluding meningeal tumors). The most common sites of occurrence were abdomen and pleura; these tumors were larger than those occurring in the extremities, head and neck or trunk, but did not demonstrate significant outcome differences. Overall 5-and 10-year metastasis-free rates were 74 and 55%, respectively, while 5-and 10-year disease-specific survival rates were 89 and 73%. Patient age, tumor size, and mitotic index predicted both time to metastasis and disease-specific mortality, while necrosis predicted metastasis only. A risk stratification model based on age, size, and mitotic index clearly delineated patients at high risk for poor outcomes. While small tumors with low mitotic rates are highly unlikely to metastasize, large tumors Z15 cm, which occur in patients Z55 years, with mitotic figures Z4/10 high-power fields require close follow-up and have a high risk of both metastasis and death.

Background To retrospectively evaluate outcomes in patients with cutaneous angiosarcoma of the face/scalp treated curatively with surgery, radiation therapy (RT), or a combination of surgery and RT. Methods 70 patients with non-metastatic angiosarcoma underwent surgery, RT, or combined-modality therapy. Of these, 20 (29%) were treated with surgery alone, 27 (39%) with RT alone, and 23 (33%) with combined-modality therapy. 44 patients received chemotherapy, either neo-adjuvantly or adjuvantly or both. Results Median follow-up was 2.1 years. The overall survival (OS) rate was 43% at 5 years, and disease specific survival was 46% at 5 years. Tumor size > 5 cm and satellitosis were prognostic for inferior OS and DSS. Combined-modality therapy (vs. surgery alone or RT alone) was associated with improved OS, DSS, and local control (LC). Conclusion Primary local therapy with combined-modality therapy was associated with improved LC, OS, and DSS for patients with angiosarcoma of the face/scalp.

Synovial sarcoma (SS) is defined by the hallmark SS18-SSX fusion oncoprotein, which renders BAF complexes aberrant in two manners: gain of SSX to the SS18 subunit and concomitant loss of BAF47 subunit assembly. Here we demonstrate that SS18-SSX globally hijacks BAF complexes on chromatin to activate an SS transcriptional signature that we define using primary tumors and cell lines. Specifically, SS18-SSX retargets BAF complexes from enhancers to broad polycomb domains to oppose PRC2-mediated repression and activate bivalent genes. Upon suppression of SS18-SSX, reassembly of BAF47 restores enhancer activation, but is not required for proliferative arrest. These results establish a global hijacking mechanism for SS18-SSX on chromatin, and define the distinct contributions of two concurrent BAF complex perturbations.

Background Hemangiopericytomas and malignant solitary fibrous tumors (HPC/SFT) are rare, closely related sarcomas with unpredictable behavior that respond infrequently to chemotherapy. An optimal systemic treatment strategy for advanced HPC/SFT has not yet been identified. Methods We retrospectively analyzed the records of 14 patients with histopathologically confirmed HPC/SFT who were treated at The University of Texas MD Anderson Cancer Center from May 2005 to June 2007. All patients were treated with temozolomide 150 mg/m2 orally on days 1-7 and days 15-21 and bevacizumab 5 mg/kg intravenously on days 8 and 22, repeated at 28-day intervals. Computer tomographic assessment of tumor size and density (Choi criteria) was used to determine the best response to therapy. The Kaplan-Meier method was used to estimate progression-free survival. Results The median follow-up period was 34 months. Eleven patients (79%) achieved a Choi partial response, with a median time to response of 2.5 months. Two patients (14%) had stable disease as the best response, and one patient (7%) had Choi progressive disease as the best response. The estimated median progression-free survival was 9.7 months with a 6-month progression-free rate of 78.6%. The most frequently observed toxic effect was myelosuppression. Conclusion Combination therapy with temozolomide and bevacizumab is a generally well-tolerated and clinically beneficial regimen for HPC/SFT patients. Additional investigation in a controlled, prospective trial is warranted.

With many novel targeted therapies currently in development for soft tissue sarcomas, a better understanding of the molecular pathogenesis and aberrations of HPC/SFT is needed to determine optimal therapeutic agents. Identifying appropriate targets and designing rational prospective clinical trials will not only improve treatment of HPC/SFT but will also lead to a new paradigm of personalized, targeted therapy.