Case Report
Primary non-metastatic angiosarcoma of the spleen
Abstract
Background: Primary splenic angiosarcoma is an extremely rare, highly aggressive malignancy hypothesised to be originating from the splenic vascular endothelium. Splenectomy is the mainstay of treatment but majority of the cases will have already metastasized at the time of presentation and hence they carry a very dismal prognosis. We wish to report a non metastatic primary splenic angiosarcoma.
Case report: A 69-year-old Asian female with past history of hypothyroidism on thyroxine presented with complaints of fatigue and vague left upper abdominal pain and fullness. Splenomegaly on palpation was confirmed by imageology. Laboratory investigations revealed anemia. She underwent splenectomy through an open laparotomy, histopathology confirming angiosarcoma. Post operative positron emission tomography (PET) scan revealed no sites of metastases. She is currently undergoing adjuvant chemotherapy.
Conclusions: Early detection and splenectomy prior to a splenic rupture is the best chance for maximizing survival, as spontaneous or traumatic splenic rupture carries the worst prognosis, with a high likelihood of immediate mortality due to hypovolemia. Benefit of adjuvant chemotherapy and radiotherapy yet to be proven.
Case report: A 69-year-old Asian female with past history of hypothyroidism on thyroxine presented with complaints of fatigue and vague left upper abdominal pain and fullness. Splenomegaly on palpation was confirmed by imageology. Laboratory investigations revealed anemia. She underwent splenectomy through an open laparotomy, histopathology confirming angiosarcoma. Post operative positron emission tomography (PET) scan revealed no sites of metastases. She is currently undergoing adjuvant chemotherapy.
Conclusions: Early detection and splenectomy prior to a splenic rupture is the best chance for maximizing survival, as spontaneous or traumatic splenic rupture carries the worst prognosis, with a high likelihood of immediate mortality due to hypovolemia. Benefit of adjuvant chemotherapy and radiotherapy yet to be proven.
