Angiosarcoma with malignant peripheral nerve sheath tumour developing in a patient with klippel-trénaunay-weber syndrome.
OBJECTIVE
We discuss the coexistence of Klippel-Trénaunay-Weber syndrome with various malignancies, the possible histogenetic pathways and therapeutic implications.


METHODS
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.


CONCLUSIONS
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.
Department of Radiology Radboud University Medical Centre Nijmegen Post-box 9101 Nijmegen NL-6500 HB The Netherlands.
Marieke J M
MJ
Ploegmakers
Maciej
M
Pruszczynski
Jacky
J
De Rooy
Benno
B
Kusters
René P H
RP
Veth