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Sarcoma
Volume 9, Issue 3-4, Pages 137-140
http://dx.doi.org/10.1080/13577140500353743

Angiosarcoma With Malignant Peripheral Nerve Sheath Tumour Developing in a Patient With Klippel–Trénaunay–Weber Syndrome

1Department of Radiology, Radboud University, Medical Centre Nijmegen, Post-box 9101, Nijmegen NL-6500 HB, The Netherlands
2Department of Pathology, Radboud University, Medical Centre Nijmegen, Post-box 9101, Nijmegen NL-6500 HB, The Netherlands
3Department of Orthopaedics, Radboud University, Medical Centre Nijmegen, Post-box 9101, Nijmegen NL-6500 HB, The Netherlands

Received 29 August 2005

Copyright © 2005 Hindawi Publishing Corporation. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Purpose: We discuss the coexistence of Klippel–Trénaunay–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–Trénaunay–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 postoperatively.

Discussion: This case describes an angiosarcoma with malignant peripheral nerve sheath tumour developing in a patient with Klippel–Trénaunay–Weber syndrome. A case never described before in literature and probably, as in our case, difficult to diagnose at first biopsy.