Primary splenic angiosarcoma presenting with unexplained anemia in a young adult: a case report

Primary splenic angiosarcoma (PSA) is an exceedingly rare and aggressive vascular malignancy originating from endothelial cells of the splenic sinusoids with rapid progression and high metastatic potential underscoring the critical need for timely diagnosis. Its nonspecific early symptoms, such as fatigue, anemia, and abdominal pain, often lead to misdiagnosis or delayed recognition, while its imaging features can mimic benign conditions like hemangioma.

We report the case of a 25-year-old Middle Eastern male who presented with progressive fatigue, anemia, and abdominal pain. Imaging revealed marked hepatosplenomegaly with multiple hypodense splenic lesions and hepatic involvement. Initial bone marrow examination demonstrated normocellular trilineage hematopoiesis with compensatory erythroid hyperplasia and no evidence of hematologic malignancy. CT-guided splenic biopsy confirmed angiosarcoma with immunohistochemical positivity for CD31, CD34, and FLI-1. The clinical course was complicated by splenic rupture and hemoperitoneum following biopsy, necessitating emergency splenectomy. The patient stabilized postoperatively and was referred for systemic oncologic therapy. He subsequently received combination chemotherapy with ifosfamide and doxorubicin, achieving initial radiologic disease stability. However, treatment was complicated by ifosfamide-induced neurotoxicity, leading to discontinuation of ifosfamide. Follow-up positron emission tomography/computed tomography (PET/CT) demonstrated no metabolically active disease, and the patient was transitioned to second-line therapy with weekly paclitaxel, with favorable clinical response and ongoing disease stability.

This case highlights the diagnostic complexity of PSA in young adults presenting with unexplained anemia and splenic lesions. Bone marrow findings may be misleadingly reactive, underscoring the importance of histopathological confirmation of splenic tissue. Early recognition and prompt surgical intervention remain critical given the aggressive nature and poor prognosis of this malignancy.