Kim Na Rae, Lee Woon Kee, Lee Jong In, Cho Hyun Yee
Department of Pathology, Gachon University Gil Medical Center, Incheon, Korea.
Korean J Pathol. 2012 Dec;46(6):590-4. doi: 10.4132/KoreanJPathol.2012.46.6.590. Epub 2012 Dec 26.
Myeloid sarcoma is a rare extramedullary myeloid tumor, which is frequently misdiagnosed when no evidence of leukemia is initially observed. Here, we report on a peculiar case of a 49-year-old man afflicted with multiple masses in the jejunum, the superior mesentery, and the serosa of the transverse colon, without leukemic manifestation. The tumor was composed of undifferentiated small round cells containing eosinophilic cytoplasm, which were negative for myeloperoxidase, nonspecific esterase, lysozyme, terminal deoxynucleotidyl transferase, leukocyte common antigen, CD3, CD4, CD15, CD20, CD30, CD43, CD56, CD68/PG-M1, CD79a, human melanoma black-45, c-kit, and CD34 with positivity only for CD68/KP1, CD99, and vimentin. Under electron microscopy, those cells had abundant membrane-bound cytoplasmic granules that measured 200 to 300 nm in diameter, which were consistent with granulocytic azurophilic granules. The tumor was finally diagnosed as a myeloid sarcoma. The presence of non-leukemic myeloid sarcomas showing immunonegativity for conventional myeloid-leukemic markers necessitated a diagnosis by ultrastructural observation.
髓系肉瘤是一种罕见的髓外髓性肿瘤,在最初未观察到白血病证据时常常被误诊。在此,我们报告一例特殊病例,一名49岁男性,空肠、肠系膜上和横结肠浆膜出现多个肿块,无白血病表现。肿瘤由含有嗜酸性细胞质的未分化小圆形细胞组成,这些细胞髓过氧化物酶、非特异性酯酶、溶菌酶、末端脱氧核苷酸转移酶、白细胞共同抗原、CD3、CD4、CD15、CD20、CD30、CD43、CD56、CD68/PG-M1、CD79a、人黑色素瘤黑色45、c-kit和CD34均为阴性,仅CD68/KP1、CD99和波形蛋白呈阳性。电镜下,这些细胞有丰富的膜结合细胞质颗粒,直径为200至300纳米,与粒细胞嗜天青颗粒一致。该肿瘤最终被诊断为髓系肉瘤。对于传统髓系白血病标志物呈免疫阴性的非白血病性髓系肉瘤,需要通过超微结构观察来进行诊断。