Epithelioid Angiosarcoma of Deep Soft Tissue

Fletcher, Christopher D.�M.; Beham, Alfred; Bekir, Songul; Clarke, Andrew M.T.; Marley, N. J. E.

doi:10.1097/00000478-199110000-00001

Cited by 264 publications

(28 citation statements)

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“…Angiosarcomas (AS) are rare malignant vascular tumors, a subset of which exists predominantly as epithelioid cells and is known as EAS [7]. The epithelioid morphology broadens the differential diagnosis, making EAS more challenging to diagnose than typical AS; furthermore, EAS behaves more aggressively than other AS.…”

Section: Discussionmentioning

confidence: 99%

“…The epithelioid morphology broadens the differential diagnosis, making EAS more challenging to diagnose than typical AS; furthermore, EAS behaves more aggressively than other AS. The most common presentation of EAS is in the deep soft tissue of an extremity; males are more commonly affected, as are individuals in their 7th decade of life [6,7]. Metastasis is common, most often to lymph nodes and other sites such as the lung, bone, soft tissue, and skin; 50% of patients succumb to the disease within 3 years.…”

Section: Discussionmentioning

confidence: 99%

“…Prognosis is poor, with approximately one half of patients succumbing to the disease within 3 years [4,5,6]. While EAS is more common among older patients, with the highest incidence in the 7th decade of life, EHE generally occurs in slightly younger middle-aged adults [5,7]. However, both EHE and EAS can present over a wide age range.…”

Section: Introductionmentioning

confidence: 99%

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Cytopathologic Characteristics of Epithelioid Vascular Malignancies

et al. 2014

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Objectives: Epithelioid hemangioendothelioma (EHE) and epithelioid angiosarcoma (EAS) are rare vascular neoplasms that share many morphological characteristics on histology but demonstrate different clinical behavior. Given the many reported clinical and morphological features shared between EAS and EHE, we examined all cases of EAS and EHE diagnosed primarily on fine needle aspiration (FNA) at our three institutions that were confirmed by a tissue diagnosis. Study Design: A total of 29 cases from 25 patients were identified: 15 EHE from 11 patients and 14 EAS from 14 patients. Results: Many cytomorphological features existed on a spectrum that overlapped considerably between EAS and EHE cases. Common features between the two entities include epithelioid morphology and eccentrically placed nucleus. Intracytoplasmic lumens (ICL), a morphological feature that may suggest vascular origin, can be found in both entities but are not always present. Conclusions: Given the general absence of vascular cytomorphological features, such as ICL, the proper classification of these tumors depends on the successful use of immunoperoxidase markers such as factor VIII-related antigen, ERG, or CD31 as well as a high index of suspicion. The distinction between EAS and EHE on FNA alone is treacherous at best.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Cytopathologic Characteristics of Epithelioid Vascular Malignancies

et al. 2014

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“…15 Papanicoloau-stained material of epithelioid sarcoma shows both folded nuclei and macronucleoli. 15,16 Reports of epithelioid variants of mesenchymal tumors including angiosarcoma 17 and leiomyosarcoma 18 contain histologic descriptions and illustrations closely matching those of the epithelioid schwannoma illustrated in our first example. In addition, the cytologic features of the epithelioid variant of leiomyoma (benign leiomyoblastoma) have been described and illustrated.…”

Section: Discussionmentioning

confidence: 59%

Fine-needle aspiration of epithelioid malignant peripheral nerve sheath tumor (epithelioid malignant schwannoma)

1997

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The epithelioid variant of malignant peripheral nerve sheath tumor (MPNST), also known as malignant epithelioid schwannoma, is a relatively rare and recently characterized clinicopathologic entity. The epithelioid variant of MPNST shares many clinical features with conventional MPNST but is characterized by different histologic and cytologic features. These include a distinctive nesting pattern and an abundance of cytoplasm not seen in histology of conventional nerve sheath tumors. Cytologically, the epitheliod variant shows a propensity to cellular discohesiveness and a plasmacytoid or epitheliod appearance that is in contradistinction to the spindled appearance of the usual MPNST. Herein, we report our experience with fine‐needle aspiration (FNA) of two epithelioid malignant schwannomas and discuss the FNA cytologic differential diagnosis. Diagn. Cytopathol. 1997;17:200–204. © 1997 Wiley‐Liss, Inc.

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“…It mimics poorly differentiated carcinoma through its epithelial appearance. Epithelioid angiosarcoma most often arises in the deep soft tissues of the extremities, although a variety of other primary sites, including the thyroid gland, skin, and adrenal glands, have also been reported [3, 4]. However, bone is a very rare origination site for this malignancy [5–7].…”

Section: Introductionmentioning

confidence: 99%

Aggressive clinical course of epithelioid angiosarcoma in the femur: a case report

et al. 2014

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BackgroundEpithelioid angiosarcoma is a rare variant of angiosarcoma, and is characterized by an epithelioid morphologic appearance that mimics carcinoma. These tumors usually arise in extraskeletal sites; origination in bone is rare.Case presentationA 69-year-old woman presented with right knee pain. Plain radiographs and magnetic resonance imaging showed an osteolytic lesion with a large soft-tissue extension into the distal femur. Under a diagnosis of metastatic carcinoma of unknown origin based on the biopsy specimen, resection and replacement with an artificial joint were performed. Histologic analysis of the resected material confirmed epithelioid angiosarcoma, supported by immunoexpression of cytokeratins and vascular markers. Three months after surgery, metastasis to the bone and lymph nodes was observed, and the patient died of the disease shortly thereafter.DiscussionEpithelioid angiosarcoma of bone is characterized by an aggressive clinical course. A possibility of epithelioid angiosarcoma of bone should be considered in cases with such epithelial features, particularly if only small specimens are available.

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Epithelioid Angiosarcoma of Deep Soft Tissue

Cited by 264 publications

References 0 publications

Cytopathologic Characteristics of Epithelioid Vascular Malignancies

Cytopathologic Characteristics of Epithelioid Vascular Malignancies

Fine-needle aspiration of epithelioid malignant peripheral nerve sheath tumor (epithelioid malignant schwannoma)

Aggressive clinical course of epithelioid angiosarcoma in the femur: a case report

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