Epithelioid angiosarcoma

Fetal fibroblasts myometrium vascular smooth muscle fetal myocardium vascular endothelial cells selected reticulum cells of lymphatic organs some CD30+ lymphoid cells (rarely) selected leiomyosarcomas, epithelioid hemangioendotheliomas, epithelioid angiosarcomas, primitive neuroectodermal tumors and Ewing s sarcomas, malignant peripheral nerve sheath tumors, and clear cell sarcomas... 

KER, keratin, as detected by a mixture of CAM5.2, MAK-6, and AEl /AE3 EMA, epithelial membrane antigen VIM, vimentin DES, desmin MSA, muscle-specific actin SMA, smooth muscle (alpha isoform) actin CALD, h-caldesmon S-IOOP, S-100 protein TY, tyrosinase Ml, MART-1 (melan-A) OGN, osteocalcin EPS, epithelioid sarcoma EPSS, epithelioid synovial sarcoma EAS, epithelioid angiosarcoma EMPNST, epithelioid malignant peripheral nerve sheath tumor CCS, clear cell sarcoma SEFS, sclerosing epithelioid fibrosarcoma ... 

Keratins are regularly found in synovial sarcoma, chordoma, parachordoma, epithelioid sarcoma, and adamantinoma. They are seen less commonly in leiomyosarcoma, malignant peripheral nerve sheath tumors, epithelioid angiosarcoma, and polyphenotypic tumors such as PNET. 

Immunoreactivity patterns may vary somewhat in epithelioid angiosarcoma as compared with classical forms of that tumor. In particular, keratin reactivity is much more often seen in the former group. However, CD31 and FLI-1 are sensitive and specific markers of epithelioid vascular malignancies as well as other endothelial... 

The potential presence of CD34 in diverse polygonal-cell soft tissue tumors, including epithelioid LMS, epithelioid MPNST, clear cell sarcoma, and epithelioid sarcoma, has diminished its potential utility as an indicator of endothelial differentiation." " We also have observed that epithelioid angiosarcomas may be reactive... 

FIGURE 4.23 Reactivity with Ulex europaeus I agglutinin in epithelioid angiosarcoma. 

FIGURE 4.22 Epithelioid angiosarcoma is potentially confused diagnostically with metastatic malignant melanoma or poorly differentiated carcinoma in the soft tissues. 

The ability of melanoma to simulate the appearance of various soft tissue sarcomas is also well documented. These include malignant peripheral nerve sheath tumors, gastrointestinal stromal tumors, epithelioid angiosarcoma, rhabdoid tumors, osteosarcomas, and primitive neuroectodermal tumors, to name a few. The detailed immunophenotypic properties of those lesions are provided elsewhere in this book. However, none of them manifests reactivity for gplOO-related melanocytic markers or tyrosinase, making those markers essential to the differential diagnostic process. 

Eusebi V, Carcangiu ML, Dina R, Rosai J. Keratin-positive epithelioid angiosarcoma of thyroid. A report of four cases. Am J Surg Pathol. 1990 14 737-747. 

Fletcher CD, Beham A, Bekir S, et al. Epithelioid angiosarcoma of deep soft tissue a distinctive tumor readily mistaken for an epithelial neoplasm. Am J Surg Pathol. 1991 15 915-924. 

Goldblum JR, Rice TW. Epithelioid angiosarcoma of the pulmonary artery. Hum Pathol. 1995 26 1275-1277. 

McCluggage WG, Clarke R, Toner PG. Cutaneous epithelioid angiosarcoma exhibiting cytokeratin positivity. Histopathology. 1995 27 291-294. 

Epithelioid angiosarcomas often show expression of keratin with both AE1/AE3 and CAMS.2, emphasizing the need to use the vascular panel to avoid diagnostic pitfall. 

Epithelioid variants of angiosarcoma are recognized as clinicopathologically distinct from epithelioid hemangioendothelioma, and they may involve the deep soft tissues. Other differential diagnostic considerations in such cases may include amelanotic melanoma, poorly... 

Angiosarcoma CD31-r/FLI-1-r epithelioid sarcoma CD34-I- synovial sarcoma negative for CD31 /CD34/FLI-1. 

FIGURE 4.32 Granular cell angiosarcoma Is an uncommon lesion with a differential diagnosis that Includes granular cell tumor, metastatic melanoma, and epithelioid leiomyosarcoma. 

Von Hochstetter AR, Meyer VE, Grant JW, et al. Epithelioid sarcoma mimicking angiosarcoma The value of immunohisto-chemistry in the differential diagnosis. Virchows Arch A Pathol Anat Histopathol. 1991 418 271-278. 

Vascular tumors Adults. Soft tissue and various visceral organs. Angiosarcoma epithelioid and spindle cell tumor, vasoformative areas epithelioid in hemangioendothelioma FVIII+, CD31+, CD34+, FLI1+, thrombomodulin+, patchy keratin+ EM to identify endothelial cells rarely required... 

Penile cancer is a rare tumour in Europe with an incidence of 1 per 100,000 men per year (Busby and Pettaway 2005). As illustrated in Chapter 13, it is most commonly diagnosed in the 6th and 7th decades of life, but can occur at any age, including childhood. Squamous cell carcinoma is by far the most common malignant disease of the penis, accounting for more than 95% of cases. Malignant melanomas and basal cell carcinomas are less common. Mesenchymal tumours such as Kaposi s sarcoma, angiosarcoma, and epithelioid haemangioendothelioma are very uncommon, with an incidence rate of less than 3%. 

Locker GY, Doroshow JH, Swelling LA, et al (1979) The clinical features of hepatic angiosarcoma a report of four cases and a review of the English literature. Medicine 58 48-63 Mann JR, Kasthuri N, Raafat F (1990) Malignant hepatic tumours in children incidence, clinical features and aetiology. Paediatr Perinat Epidemiol 4 276-289 Miller WJ, Dood GD III, Federle MP, et al (1992) Epithelioid... 

Primary hepatic malignant neoplasms may develop from hepatocytes, bile duct epithelium, endothelial cells, or lymphoid cells. Most primary malignant hepatic neoplasms are epithelial in origin, such as hepatocellular carcinoma and cholangiocarcinoma. Mesenchymal tumors such as angiosarcoma and epithelioid hemangio-endothelioma, and other sarcoma and lymphomas are rare and represent a minority of primary hepatic neoplasms (Table 17.1). 

This chapter will review rare primary malignant neoplasms such as hepatoblastoma, arising from hepatocytes cystoadenocarcinoma, arising from biliary cells angiosarcoma and other sarcomas as well as epithelioid hemangioendothelioma, arising from mesenchymal tissue and finally primary lymphoma, arising from lymphomatous tissue. 

Vascular tmnors Angiosarcoma Hemangiosarcoma Epithelioid hemangioendothelioma Leiomyosarcoma Fibrosarcoma Embryonal sarcoma Fibrous histiocytoma Lymphoma (Hodgkin s disease) Lymphoma (non-Hodgkin s disease)... 

Ishak KG, Sesterhenn lA, Goodman ZD,et al (1984) Epithelioid hemangioendothelioma of the liver a clinicopathologic and follow-up study of 32 cases. Hum Pathol 15 839-852 Itai Y, Teraoka T (1989) Angiosarcoma of the liver mimicking cavernous hemangioma on dynamic CT. J Comput Assist Tomogr 13510-912... 

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