Fine-needle aspiration cytology of epithelioid angiosarcoma: A diagnostic dilemma

Abstract

A 28-year-old woman with a 2-yr history of unilateral chronic leg swelling, initially thought to be secondary to deep vein thrombosis, later thought to be due to congenital venous malformation, eventually developed a pelvic mass, which was biopsied by fine-needle aspiration. On the basis of cytologic features on smears, high-grade sarcoma was reported. The patient underwent surgery to resect the pelvic mass, which showed anastomosing vascular channels arising from external iliac vein in histology. However, the tumor cells unexpectedly showed strong and diffuse immunohistochemical expression of cytokeratin and epithelial membrane antigen. The case was sent for expert consultation, and the expert's opinion was epithelioid angiosarcoma. The expert's diagnosis was confirmed 2 yr later by local recurrence. The clinical presentation, cytology, histology, and immunohistochemistry of the current case and 15 other cases of epithelioid angiosarcoma found in the cytology literature are summarized. This case illustrates that morphology with clinicopathologic correlation tends to be a better guide than available special techniques.

Cytopathologic Characteristics of Epithelioid Vascular Malignancies

“…Mitotic figures were readily identified. Other case reports described metachromatic matrix material [12]. The presence of mitotic figures may be more suggestive of EAS versus lower-grade tumors, but they do not appear to be a consistent characteristic among EAS.…”

“…At least one third can be positive for pancytokeratin, which complicates the exclusion of carcinoma. Furthermore, no vascular marker is entirely specific, suggesting that multiple markers should be used in combination [10,11,12,13,14]. The high-grade nature of EAS helps exclude this entity from other vascular tumors, as the majority have a high proliferation index (>10% MIB-1) and necrosis may be present [5].…”

Fine Needle Aspiration of Metastatic Epithelioid Angiosarcoma

“…Moreover, several epithelial cell markers may give positive results, such as cytokeratins AE1-AE3, epithelial membrane antigen and Cam 5.2. [7][8][9] This implies that if the epithelial markers are used alone in the absence of a clarifying clinical history, a false diagnosis of carcinoma may be made and an erroneous treatment may be initiated. This paper introduces 2 new cases of metastatic EA in which the lesion was correctly diagnosed by fine needle aspiration cytology (FNAC), illustrates their…”

Femoral artery angiosarcoma presenting with distal embolization: Report of a case

“…[6][7][8] The histopathological diagnosis remains crucial in confi rming this condition which is identifi ed by positive immunohistochemical studies for the endothelial markers CD31, CD34, factor VIII, and Fli-1. 1,[3][4][5][6][7]12 The presence of CD31 and Fli-1 confi rms an intimal origin, whilst CD31 is highly indicative of vascular malignancy. 1,7,12 Epithelioid markers, cytokeratins AE1 and AE3, are seen in about 50% of reported cases.…”

“…1,[3][4][5][6][7]12 The presence of CD31 and Fli-1 confi rms an intimal origin, whilst CD31 is highly indicative of vascular malignancy. 1,7,12 Epithelioid markers, cytokeratins AE1 and AE3, are seen in about 50% of reported cases. 3 Epithelioid angiosarcoma remains a diffi cult malignancy to establish histopathologically due to a variable degree of expression of these different markers.…”