title: Primary histiocytic sarcoma of the spleen associated with hemophagocytosis.
abstract: We report a patient with primary histiocytic sarcoma of the spleen associated with prominent hemophagocytosis. Although thrombocytopenia, probably due to hemophagocytosis, was refractory to corticosteroid therapy, the transfusion of platelets, and splenic irradiation, partial splenic embolization was effective and facilitated splenectomy for a diagnosis. The majority of the spleen showed necrosis, but viable neoplastic cells with pleomorphic nuclei and abundant cytoplasm, showing occasional erythrocytes or leukocytes, were still discernible. The neoplastic cells expressed CD68, lysozyme, and S-100 protein, and were negative for lymphoid, myeloid, and epithelial cell markers. CD163, a monocyte/macrophage-specific molecule, was positive in only some of them. Despite multiagent chemotherapy, the patient died of the disease, showing a rapidly progressive clinical course. Although the preoperative diagnosis of primary splenic histiocytic sarcoma is difficult, it has been confirmed in patients with splenomegaly of unknown etiology that clinicolaboratory features suggestive of hemophagocytosis may be important clues suggestive to the disease. CD163 expression by neoplastic cells could be confirmed only after careful observation, because the molecule may only be seen in some of the neoplastic cells.
authors.affiliation: Department of Internal Medicine, Suzuka Kaisei Hospital, 112-1 Koh-chou, Suzuka, Mie, 513-0836, Japan. naika@kaiseihp.com.
authors.forename: Koji
authors.initials: K
authors.lastname: Oka
authors.affiliation: Department of Diagnostic Pathology, Nara Medical University, Nara, Japan.
authors.forename: Hirokazu
authors.initials: H
authors.lastname: Nakamine
authors.affiliation: Department of Pathology, Yamagata University School of Medicine, Yamagata, Japan.
authors.forename: Kunihiko
authors.initials: K
authors.lastname: Maeda
authors.affiliation: Department of Pathology, Yamagata University School of Medicine, Yamagata, Japan.
authors.forename: Mitsunori
authors.initials: M
authors.lastname: Yamakawa
authors.affiliation: Department of Pathologic Oncology, Mie University School of Medicine, Tsu, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Imai
authors.affiliation: Department of Internal Medicine, Suzuka Kaisei Hospital, 112-1 Koh-chou, Suzuka, Mie, 513-0836, Japan.
authors.forename: Kohei
authors.initials: K
authors.lastname: Tada
authors.affiliation: Department of Internal Medicine, Suzuka Kaisei Hospital, 112-1 Koh-chou, Suzuka, Mie, 513-0836, Japan.
authors.forename: Motohiro
authors.initials: M
authors.lastname: Ito
authors.affiliation: Department of Internal Medicine, Suzuka Kaisei Hospital, 112-1 Koh-chou, Suzuka, Mie, 513-0836, Japan.
authors.forename: Yasuyuki
authors.initials: Y
authors.lastname: Watanabe
authors.affiliation: Department of Internal Medicine, Suzuka Kaisei Hospital, 112-1 Koh-chou, Suzuka, Mie, 513-0836, Japan.
authors.forename: Hikoji
authors.initials: H
authors.lastname: Suzuki
authors.affiliation: Department of Surgery, Suzuka Kaisei Hospital, Suzuka, Japan.
authors.forename: Makoto
authors.initials: M
authors.lastname: Iwasa
authors.affiliation: Department of Internal Medicine, Suzuka Kaisei Hospital, 112-1 Koh-chou, Suzuka, Mie, 513-0836, Japan.
authors.forename: Isao
authors.initials: I
authors.lastname: Tanaka
mesh_terms: D005260:Female; D006801:Humans; D008875:Middle Aged; D010587:Phagocytosis / Q000187:drug effects; D012509:Sarcoma / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000473:pathology* / Q000532:radiotherapy; D013160:Splenic Neoplasms / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000473:pathology* / Q000532:radiotherapy; D014057:Tomography, X-Ray Computed; D017211:Treatment Failure