Evidence of Benign Vascular Anomalies Becoming Malignant Angiosarcoma Indicates Need for Increased Surveillance in Patients

by Linda Kossoff •

CLINICAL QUESTION

Can benign vascular anomalies transform into highly malignant angiosarcoma (AS), and is a more aggressive biopsy/surgical approach warranted in certain cases of these anomalies?

BOTTOM LINE

Supportive evidence exists for the hypothesis that increased surveillance and an aggressive biopsy/surgical approach are warranted in patients with benign hemangioma whose size and characteristics change over time.

BACKGROUND: Angiosarcoma is a rare cancer accounting for less than 1% of all sarcomas. Cutaneous AS usually arises de novo, occasionally in the context of genetic syndromes. Although the existence of specific precursor lesions of AS has not been clearly defined, cases of AS developing at the site of irradiated hemangiomas have been reported.

STUDY DESIGN: Case study

SETTING: Unit of Maxillofacial Surgery–Head and Neck Department, University of Verona, Verona, Italy

SYNOPSIS: Researchers report on a rare case of extensive cutaneous AS in a woman who had a diagnosis of cutaneous angioma of the nose during the perinatal period, which was resolved with laser therapy post-birth. At age 38, the woman received a clinical diagnosis of venous malformation in the same location. This was treated with sclerotherapy and laser therapy without clinical benefit. Examination and testing confirmed the presence of an extensive mass with ulcerative and necrotic components, with an absence of nasal bone and cartilage involvement. Total rhinectomy with preservation of the skeletal support of the nose was performed. Histology results revealed vasoformative anastomosing proliferation lined by highly atypical stratified endothelial cells in the submucosa. The deeper tumoral component showed a more solid pattern of growth. Complementary radiotherapy and adjuvant systemic chemotherapy were administered. At one year post-surgery, the patient had no recurrences. Authors note that although there are few cases involving malignant evolution of benign vascular anomalies, the common thread in these cases was the co-existence of hemangioma, low-grade hemangioendothelioma, and high-grade AS. These findings suggest the need for increased surveillance of patients with benign hemangioma whose size and characteristics change over time.

CITATION: Roberto S, Riccardo N, Sokol S, et al. Cutaneous angiosarcoma. JAMA Otolaryngol Head Neck Surg. 2024;150:746-748.