Primary Composite Angiogenic Leiomyosarcoma?Epithelioid Angiosarcoma of the Brain

Abstract

The authors describe a primary sarcoma of the brain with immunohistochemical and ultrastructural features of leiomyosarcoma as well as epithelioid hemangiosarcoma. The leiomyosarcomatous component consisted of spindle cells with well-developed external lamina, subsarcolemmal densities composed of micro® laments, pinocytic vesicles, and abundant intermediate ® laments, and showed immunohistochemical reactions for smooth muscle actin. The epithelioid part of the tumor contained scattered cells reactive for alkaline phosphatase as well as CD31 and factor VIII. Many epithelioid cells were lipidized and remarkably similar to``stromal cells'' of a hemangioblastoma. Occasional Weibel-Palade bodies, indicating endothelial differentiation, were present in scattered neoplastic cells. There were also cells with features intermediate between endothelium, pericytes and smooth muscle cells, and undifferentiated mesenchymal cells. The brain at the periphery of sarcoma showed conglomerates of well-differentiated capillaries, telangiectasias and small dysplastic arteries, features that raise the possibility of origin of this tumor from a preexisting vascular developmental abnormality.

Metastatic Cardiac Angiosarcoma to the Lung, Spine, and Brain: A Case Report and Review of the Literature

“…5,11,14,[18][19][20] Weibel-Palade bodies can also be identified within the endothelial cells on electron microscopy. 17,20,29 Our patient's tissue pathology was consistent with the diagnosis of metastatic angiosarcoma (Figure 2).…”

“…5,14 Similarly, primary CNS angiosarcomas originate from the mesenchymal elements of the brain. [3][4][5][6]10,11,[15][16][17][18][19][20][21][22][23][24][25][26][27] . The pathologic features of angiosarcomas consist of malignant spindle cells lining vascular channels with hemorrhage and necrosis.…”

Epithelioid Angiosarcoma of the Septum Pellucidum

“…Interestingly, Lach and colleagues [4] described one case of primary composite leiomyosarcoma-epithelioid angiosarcoma of the frontoparietal region of a 37-year-old man. The epithelioid component was focally positive for factor VIII and CD31, but lacked immunohistochemical reactivity for cytokeratins AE1–AE3 and CAM5.2.…”

Primary central nervous system angiosarcoma: A case report and literature review

“…Angiosarcoma is a rare vascular malignancy (less than 1% of sarcomas) and mainly occurs in skin and soft tissues. [1][2][3] Localization in the CNS is extremely rare and only a few cases of primary brain angiosarcomas have been reported so far in the literature [4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22] (Table 1). It may occur in the CNS as a metastatic or a primary brain tumor.…”