Cutaneous angiosarcoma: update on biology and latest treatment

Abstract

Purpose of reviewThe present review aims to provide readers with the latest updates on the biology and clinical management of cutaneous angiosarcoma (cAS).Recent findingsThe genomic alteration of cAS is heterogeneous. Mutations are enriched in the mitosis-activated kinase (MAPK) pathway. Functional analysis has identified molecules that may serve as potential markers and therapeutic targets of angiosarcoma. These molecules include survivin, HSP90, FOXM1, miR-497-5p, KCa3.1, and miR210.This body of knowledge has not yet transferred to clinical practice. The mainstay of treatment for cAS remains surgery followed by postoperative radiotherapy. The efficacy of paclitaxel as an adjuvant chemotherapy is suggested.For patients with advanced cAS, paclitaxel is the treatment of choice. There are also second-line treatment options that are supported by evidence of varying strength. A multikinase inhibitor, pazopanib, has been assessed in several studies, most of which support its efficacy for angiosarcoma. Bevacizumab monotherapy may be effective for angiosarcoma. The efficacy of eribulin mesylate and trabectedin for angiosarcoma is currently being assessed. Recent publications highlighted the role of the immune system in the biology of cAS.SummaryFuture research efforts should focus on the following aspects of cAS: drug development directed at recent molecular targets, clinical trials designed specifically for patients with cAS, and the role of immunotherapy for cAS.

Angiosarcoma with Synchronous Cutaneous and Small Bowel Involvement: A Report of a Rare Presentation

“…It has been estimated that 16-46% of angiosarcoma cases present with metastatic disease or as multi-focal lesions, with hematogenous spread to lung and brain to be most common [1,9]. Factors associated with better prognosis for cutaneous angiosarcomas include having an age lower than 70 years, a lower number of affected regions, lack of metastatic disease, involvement of the face versus the scalp, negative margins upon resection, tumor size less than 5 cm, histologic growth demonstrating greater than 80% solid areas, and programmed death-ligand 1 (PD-L1) expression [8-11].…”

“…In the context of treatment, complete excision with wide margins is the treatment of choice for locally invasive disease. However, cutaneous angiosarcomas have been associated with high rates of local recurrence [11]. Due to the propensity for local infiltration, radiation therapy is also utilized to achieve local disease control [7].…”

Novel insights into endothelial cell malignancies

“…Angiosarcomas are aggressive endothelial malignancies that can occur anywhere in the body (primary angiosarcoma) or arise from lymphedema at the lower extremities or in the chest wall exposed to radiation for breast cancer (secondary angiosarcoma) [5, 7]. All clinical subtypes are characterized histologically by mitotic tumor cells that express CD31 to varying degrees and form vascular structures replete of blood extravasating into the parenchyma.…”

“…The tumor cells are locally invasive, often recognized at a distance from the primary tumor after resection with large margins, and highly metastatic at an early stage. Currently, the 5-year survival of patients with angiosarcoma is 33.5% [7]. Multiple genetic abnormalities have been described in angiosarcoma but no unifying driver mutation [5, 7].…”

“…Currently, the 5-year survival of patients with angiosarcoma is 33.5% [7]. Multiple genetic abnormalities have been described in angiosarcoma but no unifying driver mutation [5, 7]. Subsets of angiosarcomas show mutations of PTPRB coding for the protein tyrosine phosphatase receptor type B (PTBRB); of KDR or FLT4 coding, respectively, for VEGFR2 and VEGFR3; and single-nucleotide changes in PLCG1 , coding for phospholipase C, gamma 1.…”