Primary Cutaneous Epithelioid Angiosarcoma

Abstract

Epithelioid angiosarcomas are rare aggressive neoplasms that occur most frequently in deep soft tissues. Primary cutaneous lesions are rare, and there are discrepant findings in the literature regarding their behavior. In this study, we report a series of 13 cases of primary cutaneous epithelioid angiosarcoma and analyze their clinicopathologic features. The tumors arising in the conventional settings for cutaneous angiosarcoma (ie, in the head and neck region of elderly patients, and those occurring postradiation or associated with lymphedema) were excluded. Primary cutaneous epithelioid angiosarcoma occurred in adults (mean age, 66 y) with an equal sex distribution, and presented as solitary (n=10) or multiple (n=3) nodules ranging in size from 8 to 80 mm, with a predilection for the limbs (n=10). Histopathologically, the tumors comprised infiltrative sheets of atypical epithelioid cells within the dermis with or without the involvement of the subcutis. Vascular channel formation and intracytoplasmic lumina were seen, at least focally, in most cases. Mitoses were readily identified and necrosis was seen in 40% of the cases. The tumors were immunoreactive for vascular markers, with CD31 and FLI-1 offering the highest sensitivity. Pancytokeratin was positive in two thirds of the cases, and epithelial membrane antigen was positive in one-quarter of the cases. There was rare focal expression of Melan-A (n=2) and smooth muscle actin (n=3). Follow-up information was available for 11 patients. Six patients died of metastatic disease after a median follow-up of 12 months (range, 3 to 36 mo), and 1 patient died of unrelated causes. These findings suggest that primary cutaneous epithelioid angiosarcoma occurring outside the conventional settings of angiosarcoma is a highly aggressive malignant tumor with mortality rates in excess of 55% after 3 years.

Rapidly progressive and fatal case of extragenital cutaneous epithelioid angiosarcoma with visceral involvement

“…Histologic variants within CAS include epithelioid, clear cell, foam cell, signet cell, and granular cell 4 6 . Epithelioid CASs more commonly show infiltrative sheets of malignant cells in the dermis/subcutis with hemorrhage and, occasionally, necrosis (40%) 6 .…”

“…Epithelioid , defined as malignant cells with polygonal or rounded shape with abundant eosinophilic cytoplasm, large vesicular nuclei, and prominent eosinophilic nucleoli, is the best-described variant and is more commonly seen with sporadic (ie, not associated with radiation or lymphedema) CAS 6 . Epithelioid CASs more commonly show infiltrative sheets of malignant cells in the dermis/subcutis with hemorrhage and, occasionally, necrosis (40%) 6 . Immunohistochemistry shows expression of endothelial markers such as von Willebrand factor, CD34, CD31, vascular endothelial growth factor, and ERG.…”

“…The histopathologic differential for epithelioid CAS includes poorly differentiated cutaneous and metastatic carcinomas, melanoma, epithelioid sarcoma, and Kaposi sarcoma. Diagnosis relies on overall morphology and immunohistochemistry, with epithelioid CAS typically showing some degree of vasoformation and strong CD31 or ERG positivity 6 …”

Epithelioid Cutaneous Mesenchymal Neoplasms: A Practical Diagnostic Approach

“…Recognition of variable morphological patterns in DFSP is important to avoid misdiagnosis. Epithelioid variants have been described in angiosarcoma [140], myxofibrosarcoma [141], pleomorphic liposarcoma [142], and dedifferentiated liposarcoma [143]. Cutaneous non-mesenchymal neoplasms, especially, including malignant melanomas, adnexal tumors, sarcomatoid carcinomas, and metastatic carcinomas, may mimic epithelioid mesenchymal neoplasms.…”

Cutaneous Hematopathology

“…Recently, the use of the nuclear immunohistochemical stain ERG has been described as having superior sensitivity and specifi city in the diagnosis of cutaneous angiosarcoma (McKay et al 2012 ). Epithelioid angiosarcoma may express pancytokeratin and, less frequently, epithelial membrane antigen, although the exact frequency of co-expression ranges widely depending on the case series (Bacchi et al 2010 ;McCluggage et al 1995 ;Meis-Kindblom and Kindblom 1998 ;Suchak et al 2011 ). Aberrant CD30 expression has been reported in an angiosarcoma arising in an irradiated site, thereby mimicking anaplastic large cell lymphoma (Weed and Folpe 2008 ).…”