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KMID : 0360319970290020352
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Journal of Korean Cancer Research Association
1997 Volume.29 No. 2 p.352 ~ p.357
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A Case of Primary Splenic Angiosarcoma Associated with kasabach-Merritt Syndrome
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전진석
이규택/변재호/한기주/박성규/원종호/백승호/홍대식/박희숙
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Abstract
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Purpose:
@EN Primary malignant vascular neoplasms of the spleen are rare. It has been known that the prognosis was very poor and the splenectomy before rupture could increase survival. No effective chemotherapeutic protocol for angiosarcomas has yet to be
established but patients with or without metastatic disease may be treated by chemotherapy.
@ES Material and Methods:
@EN We experienced a case of primary splenic angiosarcoma in a 42-year-old woman with multiple purpuric skin rashes associated with consumptive coagulopathy: the Kasabach-Merritt syndrome. The CT showed spleen is diffusely enlarged and
inhomogenously
enhanced with multiple metastasis in the liver. The splenectomy was done and angiosarcoma was diagnosed. We treated her with conventional combination chemotherapy and obtained partial response. For additional response, high-dose chemotherapy and
stem
cell rescue with autologous peripheral blood stem cell transplantation was done.
@ES Result:
@EN After splenectomy, platelet count return to normal. The follow up abdominal CT scan after treatment showed complete disappeared multiple metastatic lesions in the both lobe of liver and the patient has continued to do well four months
following
discharge.
@ES Conclusion:
@EN We herein report our experience of a splenic angiosarcoma whose multiple hepatic metastases responded well to the high-dose chemotherapy.
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KEYWORD
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