Outcomes after Definitive Treatment for Cutaneous Angiosarcomas of the Face and Scalp: Re-Evaluating the Role of Combined Modality Treatment

Abstract

<h3>Purpose/Objective(s)</h3> Cutaneous angiosarcomas of the face and scalp are rare soft tissue tumors with locally aggressive malignant potential. Previous studies have shown that combined modality treatment (CMT) of surgical resection and adjuvant radiation therapy (RT) improves outcomes. We sought to update our experience with long-term outcomes in patients with cutaneous angiosarcoma of the face and scalp treated with definitive intent. <h3>Materials/Methods</h3> We performed a retrospective review of patients treated at a tertiary cancer center for non-metastatic cutaneous angiosarcoma of the face and scalp between 1962 and 2019, with a majority (69%) since 2000. All patients received definitive local treatment either with surgery, RT, or CMT. The Kaplan-Meier method was used to estimate actuarial overall survival (OS), disease-specific survival (DSS) and local control (LC). Multivariable analyses were conducted using Cox proportional hazards model. <h3>Results</h3> Median follow-up for all patients was 33 months. Of the 143 patients, the majority were male (n=109, 76%) and had a primary lesion on the scalp (n=87, 61%). Median age was 70 years at diagnosis (IQR 64-76). Slightly less than half (n=59, 41%) of patients had multifocal disease at diagnosis, and 20 (14%) presented with nodal involvement. CMT was the most common treatment approach (n=69, 48%), followed by RT alone (n=50, 35%) and surgery alone (n=24, 17%). Forty-three percent of patients (n=61) had a local relapse, resulting in a 5-yr LC of 51%. On univariable analysis 5-yr LC was significantly worse in patients with tumors >5 cm at diagnosis (19% vs 59% <5cm, p=0.01), those with multifocal disease at presentation (35% vs 59% unifocal, p<0.005), and those treated with single modality therapy (SMT) (29% vs 75% CMT, p<0.005). These factors all remained significantly associated with poorer LC on multivariable analysis (>5cm HR 2.30 [1.27-4.16], multifocal disease HR 1.95 [1.10-3.43], SMT HR 3.74 [2.01-6.96]). The 5-yr OS and DSS were 45% and 56%, respectively. When evaluating factors associated with DSS, tumor size >5 cm (HR 2.15 [95%CI 1.20-3.88]), multifocal disease at presentation (HR 1.80 [1.02-3.18]), primary site other than the scalp (HR 0.53 [0.29-0.99]), and the use of SMT (HR 2.95 [1.59-5.50]) emerged as independent predictors of DSS. The presence of multifocal disease and large tumor size were also associated with worse OS on both univariate and multivariable analyses (HR 1.59 [1.02-2.50], HR 2.00 [1.21-3.31], respectively). The use of chemotherapy was not associated with any outcome. <h3>Conclusion</h3> Large tumor size (>5 cm) and the presence of multifocal disease are negative prognostic factors in patients presenting with cutaneous head and neck angiosarcomas. With longer follow-up, our data continues to suggest that primary local therapy consisting of CMT improves LC and DSS outcomes for these patients and should remain standard of care when feasible.