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表现为梗阻性黄疸的肝外胆管髓系肉瘤

Myeloid sarcoma of the extrahepatic bile ducts presenting as obstructive jaundice.

作者信息

González-Vela M Carmen, Val-Bernal J Fernando, Mayorga Marta, Cagigal M Luisa, Fernández Fidel, Mazorra Francisco

机构信息

Department of Anatomical Pathology, Marqués de Valdecilla University Hospital, Medical Faculty, University of Cantabria, Avda. Valdecilla s/n, ES-39008 Santander, Spain.

出版信息

APMIS. 2006 Sep;114(9):666-8. doi: 10.1111/j.1600-0463.2006.apm_491.x.

Abstract

We report a rare case of myeloid sarcoma (MS) of the extrahepatic bile ducts presenting as obstructive jaundice in a patient without leukemia at time of diagnosis. A 75-year-old female presented with a one-month history of abdominal pain and jaundice. Computerized tomography scan of the abdomen showed stenosis of the extrahepatic bile ducts. Endoscopic retrograde cholangiography disclosed an irregular narrowing of the common biliary duct, suggestive of a cholangiocarcinoma, and resection was performed. Histologic examination showed diffuse transmural infiltration of malignant cells. These cells exhibited medium-sized round nuclei with central nucleoli and eosinophilic cytoplasm, and were strongly positive for myeloperoxidase, CD68, lysozyme, CD45, CD117 (c-kit protein) and CD43. Eight months following surgery the patient presented with multiple cutaneous nodules and bone marrow trephine biopsy showed acute myelomonocytic leukemia. A literature search identified two previously reported cases of MS of the extrahepatic biliary duct. MS should be taken into consideration in the differential diagnosis of a patient with obstructive jaundice. Immunohistochemistry is essential for a correct diagnosis.

摘要

我们报告了一例罕见的肝外胆管髓系肉瘤(MS),该患者在诊断时无白血病,表现为梗阻性黄疸。一名75岁女性,有1个月的腹痛和黄疸病史。腹部计算机断层扫描显示肝外胆管狭窄。内镜逆行胆管造影显示胆总管不规则狭窄,提示胆管癌,遂行切除术。组织学检查显示恶性细胞弥漫性透壁浸润。这些细胞呈中等大小的圆形核,有中央核仁,胞质嗜酸性,髓过氧化物酶、CD68、溶菌酶、CD45、CD117(c-kit蛋白)和CD43呈强阳性。术后8个月,患者出现多个皮肤结节,骨髓穿刺活检显示急性粒单核细胞白血病。文献检索发现了2例先前报道的肝外胆管MS病例。对于梗阻性黄疸患者的鉴别诊断应考虑到MS。免疫组织化学对于正确诊断至关重要。

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