Cutaneous epithelioid angiosarcoma of the head and neck. A case report of an unusual aggressive entity with limited treatment options

Abstract

Cutaneous angiosarcoma (CA) is an uncommon malignant mesenchymal neoplasm with aggressive behavior and a high mortality rate. A rare histological variant of CA is the epithelioid variant angiosarcoma (EA), which is associated with a worse clinical outcome. We report a 74-year-old female with an 8-month history of an ulcerated, poorly defined, painless tumoral mass on her scalp, face, and neck. Her past medical history included rheumatoid arthritis. A panel of immunohistochemical markers positive for vascular markers CD31 and CD34, confirmed the diagnosis. She had an overall 9-month survival since the diagnosis was made. Comorbid diseases and tumoral size make most of CA patients ineligible surgical candidates. Limited treatment options are available and due to its accelerated progression, achieving local control and prevention of metastasis in EA is challenging. We emphasize the need for early diagnosis and a multidisciplinary approach in order to improve survival in these patients.

Cells to Surgery Quiz: October 2020

“…b. Cutaneous angiosarcoma: Cutaneous angiosarcoma is a rare malignant cancer of mesenchymal origin, making up <1% of all sarcomas (Conic et al, 2019;Gonzalez et al, 2020). It usually develops in areas of sun exposure, prior radiation therapy, or lymphedema, and most cases arise spontaneously (Conic et al, 2019;Gonzalez et al, 2020).…”

“…Cutaneous angiosarcoma: Cutaneous angiosarcoma is a rare malignant cancer of mesenchymal origin, making up <1% of all sarcomas (Conic et al, 2019;Gonzalez et al, 2020). It usually develops in areas of sun exposure, prior radiation therapy, or lymphedema, and most cases arise spontaneously (Conic et al, 2019;Gonzalez et al, 2020). It is found most often in elderly men on the head or neck and classically presents as enlarging red, blue, or purple plaques, patches, or nodules on the face or scalp, which may have ulceration and nondistinct margins (Conic et al, 2019;Gonzalez et al, 2020).…”

“…It usually develops in areas of sun exposure, prior radiation therapy, or lymphedema, and most cases arise spontaneously (Conic et al, 2019;Gonzalez et al, 2020). It is found most often in elderly men on the head or neck and classically presents as enlarging red, blue, or purple plaques, patches, or nodules on the face or scalp, which may have ulceration and nondistinct margins (Conic et al, 2019;Gonzalez et al, 2020). Metastasis to the cervical lymph nodes and more wideranging metastases are often seen, and the 5-year survival rate is <15% (Gonzalez et al, 2020).…”