Angiosarcoma With Malignant Peripheral Nerve Sheath Tumour Developing in a Patient With Klippel–Trénaunay–Weber SyndromeReportar como inadecuado




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Sarcoma - Volume 9 2005, Issue 3-4, Pages 137-140



Department of Radiology, Radboud University, Medical Centre Nijmegen, Post-box 9101, Nijmegen NL-6500 HB, The Netherlands

Department of Pathology, Radboud University, Medical Centre Nijmegen, Post-box 9101, Nijmegen NL-6500 HB, The Netherlands

Department 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 possiblehistogenetic 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 fracturinghis 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 thoughtto 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 withfoci of angiosarcoma adjacent to diffuse neurofibroma and areas with high-grade malignant peripheral nerve sheathtumour. Amputation and palliative chemotherapy were indicated, but he died of pulmonary and cerebral metastases2 months postoperatively.

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





Autor: Marieke J. M. Ploegmakers, Maciej Pruszczynski, Jacky De Rooy, Benno Kusters, and René P. H. Veth

Fuente: https://www.hindawi.com/



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