PURPOSE The impacts of radiotherapy dose and exposed cardiac volume, select chemotherapeutic agents, and age at exposure on risk for late-onset cardiac disease in survivors of childhood cancer remain unresolved. PATIENTS AND METHODS We determined the rates of severe to fatal cardiac disease in 24,214 5-year survivors in the Childhood Cancer Survivor Study diagnosed between 1970 and 1999 at a median age of 7.0 years (range, 0 to 20.9 years), with a median attained age of 27.5 years (range, 5.6 to 58.9 years). Using piecewise exponential models, we evaluated the association between cardiac disease rates and demographic and treatment characteristics. RESULTS The cumulative incidence of cardiac disease 30 years from diagnosis was 4.8% (95% CI, 4.3 to 5.2). Low to moderate radiotherapy doses (5.0 to 19.9 Gy) to large cardiac volumes (≥ 50% of heart) were associated with an increased rate of cardiac disease (relative rate, 1.6; 95% CI, 1.1 to 2.3) compared with survivors without cardiac radiotherapy exposure. Similarly, high doses (≥ 20 Gy) to small cardiac volumes (0.1% to 29.9%) were associated with an elevated rate (relative rate, 2.4; 95% CI, 1.4 to 4.2). A dose-response relationship was observed between anthracycline chemotherapy and heart failure with younger children (age ≤ 13 years) at the greatest risk for heart failure after comparable dosing. CONCLUSION These observations support advances in radiation field design and delivery technology to reduce cardiac dose/volume and should guide future treatment protocols. They also inform clinical practice guidelines for post-therapy surveillance and risk-reducing strategies.

MPNST is a rare disease and, among children, is most frequent seen in adolescents. Surgery is crucial as first-line treatment for MPNST, especially if the tumor is localized at diagnosis. In patients with non-localized MPNST, the disease remains extremely difficult to manage, and both surgery and radiotherapy are interventions that should be considered.

Purpose/Objective(s): Angiosarcoma is a rare but aggressive malignant vascular tumor often associated with prior radiotherapy. This study aimed to define prognostic factors and to identify successful therapeutic approaches in the treatment of angiosarcoma with radiation therapy (RT). Materials/Methods: Between 1964 and 2009, 41 patients with nonmetastatic, histologically proven angiosarcoma were treated with RT at the University of Florida. The median age of the patients was 67 years (range, 21 to 87 years). Sixteen of the 41 angiosarcomas were considered radiation-induced. Twenty-two patients had angiosarcomas of the head and neck, including 9 of the scalp. Fourteen angiosarcomas were located on the breast or chest wall while 5 were located on the extremities, retroperitoneum, or deep soft tissues. Thirty-one patients were treated with both surgery and RT (12 with preoperative RT and 19 with postoperative RT), whereas 10 patients were treated with RT alone. The median RT dose was 60 Gy (range, 37.5 to 76 Gy). Sixteen patients were treated with RT once daily, 7 patients were treated twice daily, and 18 patients were treated three times daily. Two patients received adjuvant chemotherapy. The median follow-up was 44 months (range, 2 to 343 months). Results: The 5-year local control and overall survival rates were 64% and 54%, respectively. Of 23 patients who relapsed, 15 had a component of local failure: 11 had an isolated local recurrence and 4 occurred in conjunction with metastatic disease. The median time to a local failure was 1.5 years from RT and the longest time interval to a local failure was 10.2 years. Predictors of 5-year local control were non-scalp primary location (92% breast vs. 80% other vs. 60% non-scalp head and neck vs. 18% scalp; p\ 0.05), tumor size # 5 cm (76% vs. 26%; p \0.01), radiation-induced tumors versus de novo angiosarcoma (92% vs. 47%; p \ 0.01), and combined-modality local therapy (78% surgery + RT vs. 30% RT alone; p\0.01). Predictors of 5-year overall survival were non-scalp primary location (79% breast vs. 69% non-scalp head and neck vs. 20% other vs. 9% scalp; p \ 0.05) and tumor size # 5 cm (69% vs. 17%; p \ 0.05). With regard to both local control and overall survival, the most favorable cohort was comprised of patients who were treated with radiotherapy three times daily combined with surgery for angiosarcoma of the breast. Conclusions: For angiosarcomas treated with radiotherapy, outcome varies widely and is most impacted by site, size, and resectability. In amenable sites, aggressive treatment with surgical resection and hyperfractionated radiation therapy may offer the best prognosis.Purpose/Objective(s): Patients with pulmonary metastases (PM) from soft tissue sarcomas (STS) have historically been treated with surgery and/or chemotherapy; however, survival is limited. Since 2001 we have treated extracranial metastases from various primary malignancies with stereotactic body radiation therapy (SBRT) resulting in outstanding local control (LC), unexpectedly favorable overal...

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