Splenic angiosarcoma: a rare cause of the acute abdomen

The purpose of this article is to highlight the central role of imaging in the evaluation of patients with splenic angiosarcoma, facilitating prompt intervention and accurate diagnosis in acute presentations of this exceedingly rare entity. We performed a narrative review of the recent literature on splenic angiosarcoma. We highlight key clinical and diagnostic parameters of the 23 documented cases of splenic angiosarcoma since December 2022. We also review three cases of splenic angiosarcoma from our institution that demonstrate their clinical relevance in patients presenting with acute abdomen. Splenic angiosarcoma is a rare malignancy with few cases documented in the literature. Patients frequently present with acute left upper quadrant pain and palpable splenomegaly. Radiologically, splenic angiosarcoma is characterized by diffuse splenomegaly and a large, heterogeneously enhancing splenic mass or masses with poor demarcation and typically no calcifications. Treatment includes splenectomy along with adjuvant chemotherapy or radiotherapy, but the prognosis remains incredibly poor despite these options. Splenic angiosarcoma is a highly aggressive neoplasm that can be closely mimicked by other benign and malignant splenic lesions. Because it is rare, it is often not considered in the differential diagnosis of acute abdomen presentations. Nonetheless, it should be suspected when imaging demonstrates features such as large splenic masses, splenic tears or rupture, or metastatic disease. Its poor prognosis and strong propensity for metastasis make timely diagnosis and intervention critical to improving patient outcomes. Computed tomography, along with other imaging modalities, plays a central role in detecting splenic rupture, identifying metastases, and assessing prognostic factors that inform clinical decision‑making.