Epithelioid Angiosarcoma: A Brief Diagnostic Review and Differential Diagnosis

Abstract

Epithelioid angiosarcoma is a highly aggressive endothelial cell malignancy, most commonly arising in the deep soft tissues, but a variety of primary sites, including the adrenals, thyroid, skin, and bone, are encountered. On hematoxylin-eosin–stained sections, the pathologist encounters sheets of large, mildly to moderately pleomorphic epithelioid cells, with abundant eosinophilic cytoplasm, vesicular nuclei, and prominent nucleoli. Obvious vasoformative foci may not be present, creating confusion with metastatic carcinomas, malignant mesothelioma, melanoma, anaplastic lymphoma, epithelioid peripheral nerve sheath malignancies, and epithelioid sarcoma. Moreover, malignancies with apparent vascular differentiation must be distinguished from less aggressive vascular neoplasms, including epithelioid hemangioendothelioma. Given the range of clinical presentation, the diversity of primary sites, and the nonspecific initial histopathologic appearance, here we review the histologic findings and immunohistochemical profiles of epithelioid angiosarcoma and neoplasms in its differential diagnosis.

Spindle cell lesions of the breast: a diagnostic approach

“…Angiosarcoma of the breast displays a range of morphological appearances ranging from low-grade lesions with well-developed, interconnecting, vascular spaces to poorly differentiated tumours with little or no distinguishing features on H&E. Tumour cells are positive with a range of endothelial markers including CD31, CD34, D2-40, and ERG [58]. CK positivity may be seen in angiosarcoma, mainly in the epithelioid variant [59] which is not included in the differential diagnosis of BSCL. Angiosarcoma is negative for CD10 which may occasionally be seen in MBC.…”

Epithelioid angiosarcoma of the skin. A malignant tumor mimicking many different neoplasms

“…EA prognosis in any location is poor. Two or three years after diagnosis, around 50% die from this tumor, and 20-30% are alive and free of disease [8]. Based upon the cases we observed, patients are disease-free for less than six months.…”

Indurated purple plaques on the scalp

“…Microscopically, these cells appear large with abundant eosinophilic cytoplasm, central vesicular nuclei, and prominent nucleoli. The tumors most frequently arise in deep soft tissues, but cutaneous and visceral lesions have also been reported [5]. Cutaneous epithelioid angiosarcoma is more common in males and generally occurs later in life, with the highest incidence occurring in a patient's seventh decade of life, as presented in this case [6].…”

“…Epithelioid angiosarcoma often metastasizes early, particularly to the lungs, bone, soft tissue, and skin. Adverse prognostic factors include advanced age, tumor size greater than 5cm, bleeding, and an increased proliferative index [5]. Treatment options typically consist of surgical resection of the primary tumor, with any need for adjuvant radiotherapy or chemotherapy determined on an individual basis.…”