Primary Splenic Angiosarcoma with Liver Metastasis Caused by Malign Transformation of Hemangioma: A Case Report and Lite
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CASE REPORT
Primary Splenic Angiosarcoma with Liver Metastasis Caused by Malign Transformation of Hemangioma: A Case Report and Literature Review Hasan Sözel 1
&
Fatih Yılmaz 2
Accepted: 5 November 2020 # Springer Science+Business Media, LLC, part of Springer Nature 2020
Introduction
Case Presentation and Management
Primary splenic angiosarcoma(PSA) is an extremely rare (annual incidence ranges from 0.14 to 0.25 cases per million persons) and aggressive nonlymphoid neoplasm with a poor prognosis and a high metastasis rate, regardless of the treatment regimen, and firstly described in 1879 by Langhans.[1] These tumors often present in late-stage with metastatic disease in patients older than the fifth decade with a slight male predominance [2]. Only 20% of patients with splenic angiosarcoma survive 6 months, and median survival rates range between 4 and 18 months. [3] In PSA patients, variable clinical presentations such as weight loss, abdominal masses, shortness of breath, chest-abdominal pain, fever, anemia, thrombocytopenia, gastrointestinal bleeding, splenomegaly, and spontaneous splenic ruptures have been described as well as asymptomatic patients [2–4]. There is no standard curative treatment method in this deadly disease, splenectomy is the mainstay of therapy, and systemic cytotoxic chemotherapy with paclitaxel may be another option for improving outcomes [2, 5, 6].
A 65-year-old man was admitted with a 3-month history of left upper quadrant abdominal pain, fatigue, pallor, and loss of weight and appetite. His past medical history included renal artery stenosis, atherosclerotic cardiovascular disease, hypertension, and spleen hemangioma. There was no follow-up of his spleen hemangioma detected 4 years ago by computed tomography (CT) (Fig. 1a). Physical examination revealed massive splenomegaly and was no palpable lymphadenopathy. Anemia was present (hemoglobin: 10.8 g/L); the number and distribution of the white blood cell and platelet were in reference range, and no abnormal cells were observed in a peripheral blood smear. Liver and renal function test results were in the reference range except for the mild elevation of serum alkaline phosphatase (235 IU/L, normal = 46–116 IU/ L) and lactate dehydrogenase (456 IU/L, normal = 120–246 IU/L). The coagulation profiles, folic acid, vitamin B12, tumor markers including carbohydrate antigen 19-9, carcinoembryonic antigen, and alpha-fetoprotein determinations were unremarkable. Serologic studies for hepatitis B and C, HIV, and syphilis were negative. Abdominal ultrasonography showed hepatosplenomegaly and hypo/hyperechoic lesions, approximately 3 cm in size, were observed in the spleen and liver parenchyma. On CT examination of the abdomen; a large number of hypodense lesions of approximately 2.5 cm in diameter were observed in the liver, and the spleen parenchyma was heterogeneous, with extensive hypodense areas and dens areas (Fig. 1). A preliminary diagnosis of splenic angiosarcoma was considered with ultrasonography and CT imaging findings. We performe
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