We describe a 58-year-old male with multiple histiocytic tumors in the live
r and spleen. Multiple tumors in the liver and spleen were seen by image an
alysis, and splenectomy showed a large splenic tumor with a small nodule an
d a swelling lymph node in the hilus. Histological features of the tumors i
n the liver and spleen revealed proliferation of histiocytic cells with lar
ge and clear cytoplasm and a horseshoe-shaped nucleus. Immunohistochemical
studies revealed the presence of S-100 protein and CD1a antigen in the tumo
r cells, and neither lymphocytic marker nor lysozyme was detected. No defin
ite Birbeck granules were seen ultrastructurally, thus the tumor cells coul
d be classified into Langerhans cell type without Birbeck granules. Adminis
tration of adriamycin, vincristine, cyclophosphamide and prednisolone reduc
ed size and number of the liver tumors, and the histiocytic cells could not
be detected in repeatedly biopsied tissue from Liver tumor. We present the
clinical, immunohistological and cytological features in a visceral type o
f adult Langerhans cell histiocytosis, which responded well to chemotherapy
.