Primary vascular tumors of bone: A comprehensive literature review on classification, diagnosis and treatment.

Primary vascular tumors of bone are a heterogeneous group of neoplasms, ranging from benign hemangiomas to frankly malignant epithelioid hemangioendotheliomas and angiosarcomas. Over the years, their classification has been a matter of discussion, due to morphologic similarities and uncertainty regarding biologic behavior. Over the past decade, with the development of next-generation sequencing, there has been a significant improvement in the molecular characterization of these lesions. The integration of their morphologic, immunohistochemical and molecular features has led to a better stratification, with important prognostic and therapeutic implications. Nevertheless, primary vascular bone tumors still represent a challenge for medical oncologists. Given their rarity and heterogeneity, in the last few years, there has been no significant progress in medical treatment options, so further research is needed. Here we present a review of the current knowledge regarding primary vascular tumors of the bone, correlating clinicopathologic features with tumor behavior and therapeutic approaches.

Adjuvant chemotherapy for soft tissue sarcomas: a 10-year mono-institutional experience.

PURPOSE: The role of adjuvant chemotherapy (ACT) for soft tissue sarcomas (STS) is not standard practice. We investigated effectiveness and tolerability of ACT in patients (pts) with operated high-risk STS in clinical practice. METHODS: Medical records of pts with localized STS referred to Istituto Oncologico Veneto, Padova, from January 1, 2003 to July 07, 2012 were reviewed. Data were collected for pts with high-risk STS (size ≥5 cm, high grade and stage III). For those who received ACT, regimens used, drug doses, number of cycles, toxicity, and reasons for dose reduction or treatment interruption were recorded. Disease-free survival (DFS) and overall survival (OS) were calculated with the Kaplan-Meier method. RESULTS: Out of 96 eligible pts, median age 62 years, 36 received ACT after loco-regional treatment. Median DFS was 29.6 months (95 % CI 13.2-46.0) in pts receiving ACT and 7.8 months (95 % CI 3.9-11.7) in untreated pts (p < 0.0001); median OS was 67.0 months (95 % CI 25.4-108.6) in treated and 33.7 months (95 % CI 23.3-44.2) in untreated pts (p = 0.005). Among pts receiving ACT, a significant difference in DFS was observed between pts with limb/girdle disease (median DFS 82.4 months; 95 % CI 0.0-184.7) and pts with other primary sites (median DFS 18.3 months; 95 % CI 8.0-28.5) (p = 0.052). Grade ≥3 toxicities occurred in 20 pts (20.8 %), leading to dose reductions, delays, and treatment discontinuation in five cases. There was no treatment-related death. CONCLUSION: Our data confirm benefit of ACT with regard to DFS and OS in pts with high-risk STS, greatest for limb/girdle STS.