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The Korean Journal of Pathology 2005;39(5): 356-359.
Histiocytic Sarcoma of the Spleen: A Case Report and Review of the Literature.
Jin Ho Paik, Yoon Kyung Jeon, Sung Shin Park, Hye Sook Min, Young A Kim, Ji Eun Kim, Chul Woo Kim
1Department of Pathology, Seoul National University College of Medicine, Seoul, Korea. cwkim@plaza.snu.ac.kr
2Department of Pathology, Dongguk University International Hospital, Goyang, Korea.
3Department of Pathology, Seoul City Boramae Hospital, Seoul, Korea.
ABSTRACT
True histiocytic sarcoma is an extremely rare tumor. Its clinicopathological features are not clearly understood. Here, we report the first Korean case of primary splenic histiocytic sarcoma. A 64-year-old female having refractory thrombocytopenia, anemia and splenic mass was admitted to the hospital, and received splenectomy. Grossly, spleen was enlarged up to 18 x 13 x 8 cm and occupied with multinodular masses. Microscopically, the masses were composed of atyical large cells with abudant cytoplasm and vesicular nuclei with prominent hemophagocytosis. The tumor cells were CD68 (+), S-100 protein (-), CD21 (-), CD1a (-). After splenectomy, thrombocytopenia and anemia were corrected. However two months later the symptoms recurred, and the patient died 15 months after splenectomy. This case shared the common clinicopathologic features with the several previously reported cases in other countries, represented by splenic mass formation and prominent hemophagocytosis associated with thrombocytopenia and anemia, often leading to poor outcome.
Key Words: Histiocytic sarcoma; Spleen; Thrombocytopenia