angiosarcoma with malignant peripheral nerve sheath tumour developing in a patient with klippel–trénaunay–weber syndrome血管肉瘤恶性周边神经鞘肿瘤发展klippel-trenaunay-weber综合症患者.pdfVIP

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angiosarcoma with malignant peripheral nerve sheath tumour developing in a patient with klippel–trénaunay–weber syndrome血管肉瘤恶性周边神经鞘肿瘤发展klippel-trenaunay-weber综合症患者.pdf

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angiosarcoma with malignant peripheral nerve sheath tumour developing in a patient with klippel–trénaunay–weber syndrome血管肉瘤恶性周边神经鞘肿瘤发展klippel-trenaunay-weber综合症患者

Sarcoma, September/December 2005; 9(3/4): 137–140 ORIGINAL ARTICLE Angiosarcoma with malignant peripheral nerve sheath tumour ´ developing in a patient with Klippel–Trenaunay–Weber syndrome 1 2 1 MARIEKE J. M. PLOEGMAKERS , MACIEJ PRUSZCZYNSKI , JACKY DE ROOY , 2 ´ 3 BENNO KUSTERS , RENE P. H. VETH 1Department of Radiology, 2Department of Pathology, and 3Department of Orthopaedics, Radboud University Medical Centre Nijmegen, Post-box 9101, NL-6500 HB, The Netherlands (Received 29 August 2005) Abstract ´ Purpose: We discuss the coexistence of Klippel–Trenaunay–Weber syndrome with various malignancies, the possible histogenetic pathways and therapeutic implications. Patient: We report on a 46-year-old man presenting with increasing pain and swelling of his right lower leg after fracturing ´ his fibula. Since birth he was known as having the uncommon syndrome of Klippel–Trenaunay–Weber of his right lower leg. Methods : Our patient underwent an above-knee amputation for biopsy-proven malignant vascular tumour, first thought to be a composite hemangio-endothelioma and/or angiosarcoma with lung metastases. Results: In the amputated extremity, a vascular malformation was found with tumour showing various components with foci of angiosarcoma adjacent to diffuse neurofibroma and areas with high-grade malignant peripheral nerve sheath tumour. Amputation and palliative chemotherapy were indicated, but he died of pulmonary and cerebral metastases 2 months