ABSTRACT
Primary splenic angiosarcomas (PSA) arise from splenic endothelium are rare and impose a diagnostic challenge preoperative. They can present as asymptomatic splenomegaly however; the commonest presentation is abdominal pain. The spleen can rapidly increase in size and can manifest as spontaneous rupture which would cause peritoneal dissemination of disease. Early metastasis of PSA is seen in liver, lungs, lymph nodes and gastrointestinal system. Preoperative diagnosis requires a high index of suspicion and ultrasound, contrast enhanced computerized tomography may essential to differentiate from splenic hemangioma. Splenic angiosarcoma are best treated with splenectomy with a limited disease, with care taken not to rupture and cause spillage. PSA are resistant to adjuvant radiation and chemotherapy. Mortality is high with median survival rate of only 5 months, irrespective of treatment and hence the need to diagnose before complications. Bisphosphonates, adjuvant radiation with chemotherapy have been attempted to increase disease free survival. We report a case of PSA emphasizes on early preoperative diagnosis to avoid progression of the disease.
ABSTRACT
Primary splenic angiosarcoma is a rare highly malignant hematologic neoplasms. Based on the combination of PET/CT diagnosis of primary splenic angiosarcoma: 1 case was analyzed. The diagnosis and treat-ment of primary splenic angiosarcoma and the diagnostic significance of PET/CT in this disease were studied.
ABSTRACT
Primary splenic angiosarcoma is a rare highly malignant hematologic neoplasms. Based on the combination of PET/CT diagnosis of primary splenic angiosarcoma: 1 case was analyzed. The diagnosis and treatment of primary splenic angiosarcoma and the diagnostic significance of PET/CT in this disease were studied.
ABSTRACT
PURPOSE: 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. MATERIAL AND METHODS: 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. RESULT: Afer 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. CONCLUSION: We herein report our experience of a splenic angiosarcoma whose multiple hepatic metastases responded well to the high-dose chemotherapy.