Primary splenic angiosarcoma with fever and anemia: a case report and literature review

Abstract

Primary splenic angiosarcoma is an extremely rare and aggressive neoplasm. The prognosis of this disease is dismal, and the mean survival is less than 6 months after the diagnosis. This neoplasm typically presents with abdominal pain, splenomegaly, weight loss, and spontaneous splenic rupture. Fever is a very rare presentation of splenic angiosarcoma. Here we report the case of a 64-year-old man who presented with fever and anemia. A laparoscopic splenectomy was performed and revealed splenic angiosarcoma. The postoperative course was uneventful and the patient received 5 cycles of adjuvant chemotherapy with ifosfamide plus epirubicin. He remained disease free at 9 months after surgery. This is the first case of splenic angiosarcoma with fever as the initial presentation that was treated with laparoscopic splenectomy to be reported in the English literature.

Keywords: Splenic angiosarcoma; anemia; disease free; fever; splenectomy.

Figure 1

Abdominal CT scan showing multiple irregular heterogeneous masses in the spleen.

Figure 2

Excised specimen of the enlarged spleen with a nodular appearance.

Figure 3

Microscopic view of the splenic angiosarcoma. The neoplasm was primarily composed of spindle cells that had replaced the normal red and white pulp in the spleen. Atypical vascular spaces lined by hypertrophied tumor cells were notable (H&E × 100).

Figure 4

Immunohistochemical staining of the specimen revealing strongly diffuse positivity for CD31 (× 100).

Figure 5

Specimen exhibiting focal positivity for CD34 (× 100).

Figure 6

Specimen exhibiting diffuse positivity for factor VIII (× 100).