The epidemiology and natural history of adult gliosarcomas (GSMs), as well as patient and treatment factors associated with outcome, are ill defined. Patients over 20 years of age with GSM diagnosed from 1988 to 2004 were identified in the Surveillance, Epidemiology, and End Results (SEER) database. Kaplan-Meier survival analysis and Cox models were used to examine outcomes. Similar analyses were conducted for patients diagnosed with glioblastoma (GBM) over the same time period. GSM represented 2.2% of the 16,388 patients identified with either GSM or GBM. No significant differences between GSM and GBM were identified with respect to age, gender, race, tumor size, or use of adjuvant radiation therapy (RT). Patients with GSM were more likely to have temporal lobe involvement and undergo some form of tumor resection. The most important analyzed factors influencing GSM overall survival were age, extent of resection, and use of adjuvant RT. After adjusting for factors impacting overall survival, the prognosis for GSM appears slightly worse than for GBM (HR = 1.17, 95% CI, 1.05-1.31). GSM is a rare malignancy that presents very similarly to GBM with a slightly greater propensity for temporal lobe involvement. Optimal treatment remains to be defined. However, these retrospective findings suggest tumor excision, as opposed to biopsy only, and adjuvant RT may improve outcome. Despite therapy, prognosis remains dismal and outcomes may be inferior to those seen in GBM patients.

Background The presentation, management and outcome of patients with primary cardiac sarcomas are not well defined. Furthermore, the role of adjuvant therapy has not been delineated in the management of primary cardiac sarcomas. Methods Patients with primary cardiac sarcoma and non-cardiac sarcoma, diagnosed between 1988 and 2005, were identified in the Surveillance, Epidemiology, and End Results (SEER) database. Clinical characteristics and outcomes of primary cardiac sarcoma were defined and compared to the characteristics of non-cardiac sarcomas. Univariate and multivariate methods were used to identify factors associated with primary cardiac sarcoma survival. Results Compared to non-cardiac sarcomas, primary cardiac sarcomas were found to occur in a younger age group and were more likely to present with advanced disease. Primary cardiac sarcomas were ten times more likely to be vessel-derived (e.g. angiosarcoma), comprising almost half of all cases. Median overall survival for cardiac sarcoma patients was 6 months while that of non-cardiac sarcoma patients was significantly longer at 93 months (p < .001). Furthermore, cardiac sarcoma patients who underwent surgery had a median survival of 12 months while those who did not undergo surgery had a median survival of one month (p < .001). Conclusions Cardiac sarcomas are a distinct, rare subset of soft tissue sarcomas with a very poor prognosis. Surgery continues to be the central component of successful management. Future clinical efforts should be directed at developing approaches to permit safe radical excision and, potentially, developing effective adjuvant therapy.

Objective Angiosarcoma is an aggressive malignancy with endothelial differentiation and notoriously poor prognosis despite aggressive therapy. Limited data are available to guide management decisions. To address this limitation, we present a large retrospective analysis of angiosarcoma patients treated at a single institution over a 25-year period. Methods To identify factors that impact angiosarcoma outcomes, we reviewed demographic, tumor and treatment characteristics of angiosarcoma patients evaluated at the University of Wisconsin Hospital between 1987 and 2012. Results The cohort included 81 patients diagnosed at age 19–90 yo (median 67). Fifty-five (68%) patients presented with localized disease while 26 (32%) presented with metastases. The primary sites were visceral/deep soft tissue (42%), head and neck/cutaneous (37%), breast (16%) and limbs in the setting of Stewart-Treves (5%). The 5-year overall survival (OS) was 40% with a median of 16 months. By univariate analysis, significant adverse predictors of survival included metastases at presentation, visceral/deep soft tissue tumor location, tumor size > 5 cm, tumor necrosis and the absence of surgical excision. A trend toward prolonged survival was observed with radiation therapy and for chemotherapy in patients with metastases. Age, sex, and prior radiation showed no correlation with survival. Conclusions Our large single institution series confirms the poor prognosis of angiosarcoma, supports a central role for surgical excision in management and highlights the need for novel therapies particularly in patients who present with metastatic disease.