Neurofibrosarcoma of the radial nerve in von Recklinghausen's disease with metastatic angiosarcoma.

Intraneural angiosarcoma and angiosarcoma arising in benign and malignant peripheral nerve sheath tumours: clinicopathological and immunohistochemical analysis of four cases

“…The recurent MPNST with focal angiosarcomatous differentiation (case 3) was characterized by a rather abrupt transition between both neoplastic components. Interestingly, the cerebral metastasis showed features of angiosarcomatous differentiation without nerve sheath differentiation; an identical observation was made in a previous case report 14 . As in previously reported cases 7 vascular differentiation within the reported MPNST (case 3) was characterized by a broad morphological spectrum ranging from well‐differentiated angiosarcomatous areas to solid angiosarcomatous areas.…”

Malignant mesenchymoma of ulnar nerve: combined sarcoma of nerve sheath and rhabdomyosarcoma.

“…It is thus not surprising to find heterologous tissues within mesenchymal tumours of peripheral nerve, or in a mesenchymal portion of neoplasms of mixed mesenchymal and neuroepithelial origin of the neuraxis-for example, 527 *. : Xe osteoid (White, 1971), bone (Shuangshoti and Netsky, 1971 a;Shuangshoti et al 1971 a), cartilage (Shuangshoti and Netsky, 1971 b;, benign and malignant melanocytes (Bednar, 1957;Fu et al 1975;Hahn et al 1976a,b), malignant adipose tissue (Shuangshoti, 1973;Naka et al, 1975), maligant blood vessels (Macaulay, 1978), and malignant striated muscle as in the present instance White, 1971;Woodruff et al, 1973). It is not surprising either to find neoplasms of mixed mesenchymal and neuroepithelial derivatives outside the neuraxis.…”

Metastatic angiosarcoma arising in malignant peripheral nerve sheath tumor in a young patient with neurofibromatosis type 1

“…It is of particular interest that our patient, with only a very small focus of one tumor demonstrating angiosarcoma, rapidly developed pulmonary metastatic disease with pure angiosarcoma histology. Nevertheless, this pattern of metastatic angiosarcoma leading to death in patients with angiosarcomatous differentiation of a primary MPNST has been documented in prior case studies . While gross total excision is the definitive treatment for both MPNST and angiosarcoma, in the latter malignancy there are data showing some benefit with chemotherapy, specifically in the setting of metastatic disease .…”