Clinicopathological features and prognostic factors in angiosarcoma: A retrospective analysis of 200 patients from a single Chinese medical institute

Abstract

Angiosarcoma is a rare soft tissue sarcoma, and the data about its clinicopathological features and prognostic factors are limited. The purpose of the present study was to report a large series of angiosarcoma at a single institution. Clinical data from 200 cases of angiosarcoma from the Shanghai Cancer Center (Shanghai, China) between March 2006 and March 2014 were retrospectively analyzed. The study population included 97 males and 103 females with ages between 4 and 91 years (median, 53 years). According to the tumor location, 200 cases were divided into 4 groups: i) Tumors involving the head and neck; ii) breast; iii) viscera (including internal organs and bone); and iv) soft tissue (including trunk and extremities). Of the 113 patients with follow-up data, 46 patients succumbed to the disease with a median interval of 10 months. Tumor recurrence/metastasis was identified in 66 patients with a median interval of 4 months. The disease-free survival (DFS) rate at 5-years was 19.3% and the overall survival (OS) rate at 5-years was 40.8%. Site of tumor origin, size (≥5 cm) and histological differentiation influenced DFS (P=0.032, 0.038 and <0.001, respectively), and OS (P<0.001, 0.008 and <0.001, respectively) rates. Age (<65 years) and multimodal treatment correlated with improved OS (P=0.003 and <0.001, respectively). Tumor differentiation and treatment modality were identified to be independent determinants of OS (P<0.001 and 0.038, respectively). Tumor recurrence/metastasis was an independent predictor of DFS (P<0.001). The prognosis of angiosarcoma is poor and the mortality rate is high. The site of tumor origin, size, histological differentiation, age, treatment modality and tumor recurrence/metastasis are all significant prognostic factors. In the present study, multimodal treatment may improve the clinical outcome of patients with angiosarcoma.

Keywords: angiosarcoma; clinicopathological features; prognostic factors; retrospective analysis; soft tissue sarcoma.

Figure 1.

Representative microscopic images of the appearance of angiosarcomas. Low grade angiosarcoma demonstrated (A) irregular, anastomosing vascular channels (hematoxylin and eosin staining, original magnification, ×200). (B) The ramifying channels were lined with atypical endothelial cells (hematoxylin and eosin staining, original magnification, ×400). (C) High grade angiosarcoma exhibiting proliferation of high grade spindle tumor cells without clear vasoformation (hematoxylin and eosin staining, original magnification, ×400). (D) High grade angiosarcoma with epithelioid appearance with abundant amphophilic to lightly eosinophilic cytoplasm (hematoxylin and eosin staining, original magnification, ×400).

Figure 2.

Immunohistochemical features of angiosarcoma. Tumor cells stained positively for the cluster of differentiation 31 antibody with (A) hematoxylin and eosin staining; and (B) immunohistochemical staining, original magnification, ×100. Nuclear erythroblast transformation-specific-related gene staining with (C) hematoxylin and eosin staining; and (D) immunohistochemical staining, original magnification, ×100, and Friend leukemia virus integration 1 staining with (E) hematoxylin and eosin staining; and (F) immunohistochemical staining, original magnification, ×200 were positive in tumor cells.

Figure 3.

Kaplan-Meier curves comparing DFS and OS. (A) DFS and (B) OS of patients with different tumor location. (C) DFS and (D) OS of patients with different tumor sizes. (E) DFS and (F) OS of patients with different tumor differentiation. DFS, disease free survival; OS, overall survival.