Cutaneous Epithelioid Angiosarcoma Research Articles

Misdiagnosis of Cutaneous Epithelioid Angiosarcoma as Diabetic Foot Ulcer: A Case Study

Background. AS is a malignant tumor that originates from vascular endothelial cells and is known for a high rate of local recurrence and metastasis. Case Report. A 48-year-old male presented with cutaneous epithelioid AS. Cutaneous AS of the foot is quite rare, especially in the absence of predisposing factors, and in this patient it was previously misdiagnosed as a DFU. Conclusion. Physicians should be aware of this rare presentation of cutaneous AS. The authors of the current report advise regular clinical reassessment of chronic ulcers and biopsies of nonhealing wounds, even when adequate wound treatment has been administered, with the goal of identifying ulcerated skin malignancies and preventing delay in providing appropriate treatment.

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

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

Aggressive epithelioid angiosarcoma of the scalp: a diagnostic challenge

Cutaneous epithelioid angiosarcoma is a rare malignant tumour of vascular origin, usually located in the scalp. Epithelioid angiosarcomas are mimickers of a number of poorly differentiated tumours including carcinomas, lymphomas and melanomas. We report the case of an 80 year old lady with multiple scalp swellings of 4 months duration. On further evaluation, the computerized tomography (CT) scan showed multiple well defined isodense to hyperdense soft tissue swellings on the left frontal region without any intracranial extension. A biopsy of the lesion was taken which on histopathological and immunohistochemical examination confirmed the diagnosis of epithelioid angiosarcoma. The patient was treated with adjuvant radiotherapy and on follow up has local recurrences at the same site six months after initial diagnosis. To conclude; epithelioid angiosarcoma is an aggressive tumour of elderly patients with local recurrences and poor prognosis.

The spectrum of rare morphological variants of cutaneous epithelioid angiosarcoma.

Unusual cytoplasmic alterations have recently been reported in poorly differentiated cutaneous angiosarcoma, making an accurate diagnosis challenging. As these tumours remain poorly documented, we aimed to study their clinicopathological characteristics more comprehensively. Six cutaneous angiosarcomas with unusual cytoplasmic alterations were identified from referral files. All tumours arose as nodules or plaques (range: 05-195 mm) on sun-damaged skin of the head and neck of elderly males (median age: 76.5 years). Histologically, the tumours were composed of enlarged epithelioid cells showing prominent signet ring (n = 3), foam (n = 2) or granular cell (n = 1) change. Vasoformative elements were only focally noted. By immunohistochemistry, all tumours expressed CD31 and avian v-ets erythroblastosis virus E26 oncogene homologue (ERG). Foam cell change was associated with additional expression of CD68 and CD163. Follow-up (median: 8 months) showed death from disease (n = 1), death from a gastrointestinal bleed (n = 1), and a cutaneous metastasis (n = 1). Only two patients are alive with no evidence of disease. Our findings outline the morphological spectrum of cytoplasmic change in cutaneous angiosarcoma. Awareness and a high degree of suspicion in the context of tumours affecting sun-damaged skin of the elderly are necessary to direct appropriate immunohistochemical work-up with inclusion of the endothelial cell markers CD31 and ERG.

Signet Ring Cell Angiosarcoma

We report 2 cases of cutaneous epithelioid angiosarcoma featuring predominantly signet ring cells. The patients-a woman, 68 years of age, and a man, 85 years of age, respectively-were referred for slowly growing indurated plaques on their parietal and retroauricular skin. Microscopic examination showed diffuse dermal proliferations comprising polygonal cells and relatively abundant cytoplasm. Because the tumor cells often were distended by variably sized vacuoles pushing the nuclei to the periphery, the nuclear profile tended toward a crescent-like morphology. Abortive luminal formations were recognized. The tumor cells were positive for CD31, CD34, and D2-40/podoplanin, with no expression of epithelial or melanocytic markers. In 1 case, upon ultrastructural examination of paraffin-embedded tissue-cut from wax tissue and reprocessed-the optically empty spaces were surrounded by a membrane with ultrastructural features identical to those of the outer cell membrane, suggesting that these spaces corresponded to the formation of primitive intracytoplasmic lumina within the tumor cells. A few Weibel-Palade bodies also were noted. Our report offers further evidence that epithelioid angiosarcoma of the skin has a broad microscopic spectrum and that tumors displaying a preponderant population of signet ring cells pose further diagnostic challenges. A brief overview of cutaneous malignant tumors in the differential diagnosis of signet ring cell angiosarcoma is provided.

Cutaneous epithelioid angiosarcoma: A rare aggressive neoplasm

Cutaneous epithelioid angiosarcoma: A rare aggressive neoplasm

Primary Cutaneous Epithelioid Angiosarcoma

Primary Cutaneous Epithelioid Angiosarcoma

Primary multicentric cutaneous epithelioid angiosarcoma

Cutaneous epithelioid angiosarcoma is a rare malignant vascular tumor, most commonly affecting elderly men, and is usually located on the extremities. We report a case of an 81-year-old lady who presented with two ulcerated plaques over the right temporal and parietal scalp of 1 year duration. The right submaxillary and submandibular lymph nodes were enlarged and tender. Computed tomography (CT) scan of the head showed soft tissue swelling over parietal and temporal areas and there was no intracranial extension. Ultrasonogram of the abdomen showed hyperechoic areas in liver suggestive of secondaries. Histopathology of the skin lesion showed the dermis and subcutis composed of clusters of atypical epithelioid cells with vesicular nuclei, prominent nucleoli and eosinophilic cytoplasm with increased mitotic figures. Immunohistochemical staining revealed CD-31, 33, 34 and vimentin positivity, while cytokeratin was negative confirming the diagnosis of epitheloid angiosarcoma. This case report highlights the unusual occurrence of multicentric epitheloid angiosarcoma on the scalp with secondaries in the liver.

Primary Cutaneous Epithelioid Angiosarcoma

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.

Cutaneous epithelioid angiosarcoma occurring at a peristomal site

Cutaneous epithelioid angiosarcoma occurring at a peristomal site

Fine‐needle aspiration of metastatic radiation‐induced cutaneous epithelioid angiosarcoma of the breast to the liver: A diagnostic dilemma

Radiation therapy is a significant risk factor for the development of angiosarcoma. With the increase in breast conservation treatment, the incidence of cutaneous radiation-induced angiosarcoma of the breast is rising. If the angiosarcoma demonstrates epithelioid features, the tumor cells may present a diagnostic dilemma on fine-needle aspiration cytology. We present a case of metastatic radiation-induced cutaneous epithelioid angiosarcoma of the breast to the liver and a review of the literature.

Cutaneous epithelioid angiosarcoma: a neoplasm with potential pitfalls in diagnosis

Angiosarcoma (AS) is a rare neoplasm. Cutaneous AS is the most common form of AS. The epithelioid variant of the disease, however, is a rare entity. This subset can histologically mimic non-vascular neoplasms and impose serious challenges in reaching the correct diagnosis. We present five patients with cutaneous epithelioid angiosarcoma (EAS); in none, the clinical diagnosis included a vascular lesion. Three patients had history of breast conservation surgery with/without radiation therapy. Other patients had no previous radiation, and there was no lymphedema in any of the cases. The histopathological examination of the biopsy specimens by hematoxylin and eosin method was not suggestive of a malignant vascular neoplasm initially and the differential diagnoses included carcinoma, malignant melanoma and atypical lymphoid infiltrate. Only after performing immunohistochemical studies that included vascular markers, a definitive diagnosis was possible. Some cases showed unusual histopathological features. Cutaneous EAS is a rare variant of cutaneous AS that can mimic a variety of more common, non-vascular neoplasms, creating a major pitfall in the diagnosis. A careful and thorough histopathological examination and a high index of suspicion, along with appropriate immunohistochemical evaluation, can help reach a correct diagnosis and provide optimal patient care.

Cutaneous CD30-Positive Epithelioid Angiosarcoma Following Breast-Conserving Therapy and Irradiation: A Potential Diagnostic Pitfall

A case of cutaneous epithelioid angiosarcoma with anomalous CD30 expression, occurring after breast-conserving surgery and adjuvant irradiation, is reported. This seems to be the first such case reported. The differential diagnosis of CD30 epithelioid angiosarcoma is discussed.

Primary Cutaneous Epithelioid Angiosarcoma

This article does not have an abstract.

Macrophage‐rich epithelioid angiosarcoma mimicking malignant melanoma

Cutaneous epithelioid angiosarcoma is a type of cutaneous angiosarcoma and usually arise both on the head or neck of the elderly. An 86-year-old male with an epithelioid angiosarcoma of the scalp that mimicked malignant melanoma. A large irregular dark grey-blue plaque with an adjacent speckled tan nodule was suggestive of a primary cutaneous malignant melanoma with adjacent in-transit metastasis. Both had a well-circumscribed growth pattern and were composed of numerous large epithelioid cells with scattered severe atypia and mitoses. The tumor was positive for S-100 protein and vimentin and negative for low- and high-molecular weight cytokeratins. However, at high power, the epithelioid cells with severe atypia were negative for S-100 protein, and abundant large epithelioid macrophages were responsible for the S-100 protein positivity. The malignant tumor cells were negative for HMB-45, positive for CD31 and Factor VIII-related antigen, and focally positive for CD34. A focus of infiltrative, classical angiosarcoma with irregular vascular channels lined with plump, anaplastic endothelial cells was then found deep to the epithelioid tumor. Macrophage-rich epithelioid angiosarcoma demonstrates abundant S-100 protein-positive epithelioid macrophages. This subset of epithelioid angiosarcoma may mimic malignant melanoma and may present as a pitfall in diagnosis.

Primär multiples epithelioides kutanes Angiosarkom der Hand - Ein Fallbericht und Literaturübersicht

The case of a primary multicentric cutaneous epithelioid angiosarcoma is described. A 39-year-old female patient presented with a tumor on the left distal forearm and the fourth finger of the left hand. Partial amputation of the left hand and local excision of the tumor on the left forearm were performed. For the next three years the patient was free of local or systemic recurrence. After this time period, a left shoulder exarticulation had to be performed because of tumor recurrence in the left axilla. The patient died 64 months after diagnosis. Cutaneous epithelioid angiosarcoma is a highly malignant tumor which has only been described once on the hand. The treatment primarily is a surgical one. If possible, the function of the extremity should be preserved as long as possible.

Cutaneous epithelioid angiosarcoma: A potential diagnostic trap for cytopathologists

Cutaneous epithelioid angiosarcoma: A potential diagnostic trap for cytopathologists

Cutaneous epithelioid angiosarcoma exhibiting cytokeratin positivity

Cutaneous epithelioid angiosarcoma exhibiting cytokeratin positivity

Cutaneous epithelioid angiosarcoma and endothelial markers

Cutaneous epithelioid angiosarcoma and endothelial markers

Cutaneous epithelioid angiosarcoma: a clinicopathological study of four cases.

Four cases of cutaneous epithelioid angiosarcoma are described together with the potential diagnostic trap of mistaking these tumours for poorly differentiated carcinoma or malignant melanoma. The immunophenotypic profile using four endothelial markers showed positive staining in all cases for factor VIII related antigen in a predominantly paranuclear dot-like fashion and for CD31 (JC70); in three cases for CD34 (QB-END/10) and in two cases with UEA-1. All four cases were cytokeratin (CAM 5.2 and AE1/AE3) negative in contrast to the positive staining reported at non-cutaneous sites. Aberrant S-100 protein expression was seen in one case. In two cases subsequent recurrences showed better differentiation than the original tumour. Electronmicroscopy confirmed the absence of non-endothelial lines of differentiation but failed to reveal Weibel-Palade bodies.