A very rare case of primary hemangioendothelioma of the spleen was reported. A 75 years old male patient had outstanding clinical feature of leukemoid reaction caused by some malignant tumor and was diagnosed by autopsy to have had its origin in the spleen.
Autopsy findings revealed diffuse tumor in the spleen and metastasis in the bilateral lungs, liver, bilateral kidneys, bone marrow of femur and approximately generalized lymp nodes. Histological studies proved the tumor to be a hemangioendothelioma.
The literature on this subject was reviewed.