Back to Search
Start Over
Angiosarcoma with malignant peripheral nerve sheath tumour developing in a patient with Klippel-Trénaunay-Weber syndrome.
- Source :
- Sarcoma; Sep-Dec2005, Vol. 9 Issue 3/4, p137-140, 4p
- Publication Year :
- 2005
-
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. [ABSTRACT FROM AUTHOR]
Details
- Language :
- English
- ISSN :
- 1357714X
- Volume :
- 9
- Issue :
- 3/4
- Database :
- Complementary Index
- Journal :
- Sarcoma
- Publication Type :
- Academic Journal
- Accession number :
- 106343286
- Full Text :
- https://doi.org/10.1080/13577140500353743