A case of littoral cell angioma of the spleen
演題番号 : PS-1-290
根来 暁子 （Akiko Negoro):1、佐藤 忠嗣 （Tadatsugu Sato):2、道下 雄介 （Yusuke Michishita):1、本橋 知美 （Tomomi Motohashi):1、阿部 大二郎 （Daijiro Abe):1、長谷川 直樹 （Naoki Hasegawa):3、角田 幸雄 （Yukio Kakuta):3、平澤 晃 （Akira Hirasawa):1
1:Department of Hematology, Yokohama Rosai Hospital, Yokohama, Japan、2:Transfusion Service, Yokohama Rosai Hospital, Yokohama, Japan、3:Department of Pathology, Yokohama Rosai Hospital, Yokohama, Japan
Littoral cell angioma of the spleen is extremely rare vascular tumor, first described by Folks in 1991. Here we report a case. A 59-year-old woman was indicated by chance as having anemia, thrombocytopenia and splenomegaly at a complete health checkup in December 2009, and referred to our facility. She had no clinical symptoms, but her Hb level was 9.8 mg/dL, and platelet count was 40,000 /μL. CT and MRI revealed enlarged spleen of 112 X 60 X 112 mm size with some low-attenuation nodular masses. Esophago -gastroduodenoscopy (EGD) and total colonoscopy (TCS) showed no remarkable findings, and gynecological examination only found a uterine myoma. No abnormal cells were found by bone marrow aspiration. Those levels of sIL-2R, CEA and CA19-9 were 713 U/mL, 0.7 ng/mL and 13.3 U/mL, respectively. Splenectomy was performed at July 2010 because of the progression of pancytopenia. After the procedure, her Hb level increased up to 11.4 mg/dL and platelet count recovered up to 270,000 /μL. Pathological examination of the 620g resected spleen showed many spongy nodular lesions of various sizes, max 3cm in diameter, and microscopically hyperplasia of endothelial cells in the splenic sinus with positivity of factor VIII and focal CD68. Thus, diagnosis of littoral cell angioma was established. Littoral cell angioma should be considered in differential diagnosis of multinodular splenomegaly, but primary tumors of the spleen other than lymphoid malignancy are quite rare. We will present with bibliographic consideration.