Primary splenic
angiosarcoma (PSA) is a rare
neoplasm of vascular origin associated with aggressive
behavior and poor
prognosis. The clinical presentation is usually non-specific and is mostly characterized by a
wasting disease with
anemia and
splenomegaly, mimicking a wide range of entities. The authors present the case of an 80-year-old
woman with cardiovascular comorbidities with a 6-month
history of
weight loss,
fatigue, weakness,
pallor, and
abdominal pain. The
physical examination showed massive
splenomegaly and
pallor. After a thorough evaluation that ruled out lymphoproliferative
diseases, the working
diagnosis was a myelodysplastic disorder. A few days after discharge, she returned to the
emergency room with severe
abdominal pain, worsening
fatigue, and a remarkable
pallor.
Point-of-care ultrasound showed free intraperitoneal fluid.
Spleen rupture was confirmed by abdominal computed
tomography (CT) scan, and an
emergency laparotomy with
splenectomy was performed. The
postoperative period was uneventful, and the
patient recovered in a few days. The histopathology confirmed the
diagnosis of PSA and the
patient was referred to an oncological center. Two months later staging CT demonstrated
liver and peritoneal
metastases, and despite the
chemotherapy she died 6 months after the
diagnosis