Angiosarcoma outcomes and prognostic factors: a 25-year single institution experience

Abstract

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 ages 19 to 90 years (median, 67 y). Fifty-five (68%) patients presented with localized disease, whereas 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 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.

FIGURE 1

Representative images of the histologic spectrum of angiosarcoma reviewed in this series. (a) vasoformative angiosarcoma showing well-formed irregular anastomosing vascular channels dissecting dermal collagen; the endothelial cells show mild cytologic atypia and lack mitoses; (b) vasoformative angiosarcoma with high grade nuclear features and (c) epithelioid angiosarcoma composed of solid sheet of polygonal cells resembling carcinoma; vascular channels are not seen.

FIGURE 2

Five-year overall survival in all patients.

FIGURE 3

Five-year overall survival in angiosarcoma patients based on (a) localized vs. metastatic disease, (b) visceral/deep soft tissue vs. all other anatomic sites, (c) association with prior radiation, (d) tumor size, (e) vasoformative vs. non-vasoformative architectural pattern and (f) tumor necrosis.

FIGURE 4

Five-year overall survival in angiosarcoma patients segregated by surgical resection: (a) all patients, (b) localized disease only and (c) metastatic disease only. Five-year overall survival in angiosarcoma patients segregated by radiation therapy: (d) all patients, (e) localized disease only and (f) metastatic disease only. Five-year overall survival in angiosarcoma patients segregated by chemotherapy: (g) all patients, (h) localized disease only and (i) metastatic disease only.