Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
J Korean Med Sci. 2010 Feb;25(2):313-6. doi: 10.3346/jkms.2010.25.2.313. Epub 2010 Jan 19.
Histiocytic sarcoma (HS) is a very rare neoplasm that often shows an aggressive clinical course and systemic symptoms, such as fever, weight loss, adenopathy, hepatosplenomegaly and pancytopenia. It may present as localized or disseminated disease. We describe here a 63-yr-old male who manifested systemic symptoms, including fever, weight loss and generalized weakness. Abdominal and chest computed tomography failed to show specific findings, but there was suspicion of multiple bony changes at the lumbar spine. Fusion whole body positron emission tomography, bone scan and lumbar spine magnetic resonance imaging showed multiple bone lesions, suggesting a malignancy involving the bone marrow (BM). Several BM and bone biopsies were inconclusive for diagnosis. Necropsy showed replacement of the BM by a diffuse proliferation of neoplastic cells with markedly increased cellularity (95%). The neoplastic cells were positive for lysozyme and CD68, but negative for T- and B-cell lineage markers, and megakaryocytic, epithelial, muscular and melanocytic markers. Morphologic findings also distinguished it from other dendritic cell neoplasms.
组织细胞肉瘤（HS）是一种非常罕见的肿瘤，常表现为侵袭性临床病程和全身症状，如发热、体重减轻、淋巴结病、肝脾肿大和全血细胞减少症。它可能表现为局限性或播散性疾病。我们在此描述一位 63 岁男性，其表现为全身症状，包括发热、体重减轻和全身乏力。腹部和胸部计算机断层扫描未能显示特定发现，但怀疑腰椎有多处骨改变。全身融合正电子发射断层扫描、骨扫描和腰椎磁共振成像显示多处骨病变，提示骨髓（BM）有恶性肿瘤累及。几次 BM 和骨活检均不能明确诊断。尸检显示 BM 被弥漫性增殖的肿瘤细胞所取代，细胞明显增多（95%）。肿瘤细胞对溶菌酶和 CD68 呈阳性，但对 T 细胞和 B 细胞谱系标志物、巨核细胞、上皮细胞、肌肉和黑色素细胞标志物呈阴性。形态学发现也将其与其他树突状细胞肿瘤区分开来。
