Splenic Angiosarcoma Presenting With Jaundice, Ascites and Bone Marrow Fibrosis

Abstract

A middle aged chronic alcoholic presented with deep jaundice, markedly enlarged and tender spleen with leukoerythroblastic blood picture and bone marrow biopsy showing mild fibrosis. He was tested negative for HIV, hepatitis B and C viruses. Besides very high serum bilirubin, alkaline phosphatase was raised four times the normal value. Contrast enhanced CT showed enlarged spleen and liver with multiple heterogenous lesions in spleen and tiny hypo-dense lesions in liver. In hospital, he developed haemolytic uraemic syndrome and succumed to his illness. At autopsy spleen weighed 5200 gms and variegated in appearance due to large areas of necrosis and whitish tumour nodules. Histology revealed morphology of an angiosarcoma. Liver was also infiltrated by the tumour mainly in and around portal tract areas.

Epithelioid angiosarcoma metastases to the bone marrow manifesting as erythroblastic anemia and leukocytosis: a and review of the literature

“…These unusual metastases occurred in four cases of splenic AS, two deriving from the bone, and one case each from the sinonasal region, thyroid and the liver [5][6][7][8][9][10][11][12]. Interestingly, although splenic AS makes only 4% of ASs [1], they were responsible for 44% of bone marrow-metastasizing cases [5,6,8,9]. This may indicate the special predilection of splenic AS for bone marrow involvement.…”