Analysis of clinicopathologic characteristics, misdiagnosis and missed diagnosis of 4 malakoplakia cases
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摘要:
目的 提高对软斑病（malakoplakia）的发病机制、临床病理学特征的认识及诊疗水平，避免误诊、漏诊。 方法 收集山西省肿瘤医院2007年3月至2017年3月诊断为软斑病4例患者的临床资料，观察其组织病理学形态，行免疫组织化学及特殊染色。4例患者平均发病年龄为63（56~76）岁。其中男性2例、女性2例，发生于膀胱2例、双侧输尿管1例、右侧盆腔1例。1例有糖尿病史患者合并系统性红斑狼疮，另3例无特殊疾病史。 结果 4例患者的病变组织学形态均显示大量片状分布的组织细胞、同心圆状排列的嗜碱性Michaelis-Gutmann（M-G）小体、淋巴细胞、浆细胞、嗜酸性粒细胞及中性粒细胞浸润。免疫组织化学法显示组织细胞PGM-1及CD68阳性。钙、铁染色及PAS染色中M-G小体均阳性。 结论 软斑病是一种罕见肉芽肿性病变，可在多个器官形成瘤样结节。多累及泌尿生殖系统，但在身体各器官均可发生。无特异性的临床表现，影像学多表现为占位性病变，因此极易被临床误诊为恶性病变。确诊主要依靠病理诊断，因其罕见性致使病理工作者易漏诊，其中特殊染色方法对诊断具有较大的帮助。 -
关键词:
- 软斑病 /
- Michaelis-Gutmann小体 /
- 特殊染色
Abstract:Objective To analyze the pathogenesis, clinicopathological characteristics, diagnosis, and treatment of the malakoplakia forthe purpose of avoiding misdiagnosis and missed diagnosis. Methods Clinicopathological features of four malakoplakia patients admitted to the Shanxi Cancer Hospital from March 2007 to March 2017 were studied by light microscopy, immunohistochemistry, and specific stain technology, combining with a review of literature. Results The onset age of the 4 patients(two males and two females)ranged 56-76 years(median: 63 years).Two patients had malakoplakia in bladder; one patient had malakoplakia in bilateral ureters; and one case had malakoplakia in right pelvic.One patient has a history of systematic lupus erythematosis and diabetes.Malakoplakiais characterized by the accumulation of foam cells and concentrically-layered basophilic inclusions called Michaelis-Gutmann(M-G)bodies.The foam cells were positive for CD68 and PGM-1 by immunohistochemistry, indicating its derivation from the histocyte.M-Gbodies were positive for periodic acid-Schiff reaction, iron, and calcium by specific staining technology. Conclusion Malakoplakia is arare inflammatory condition and a common urinary tract disease, but it may involve any organ with non-specific symptoms and canform tumor-like nodules that clinically simulate malignancy in various organs.Clinicopathological features can be used as diagnosticmarkers in malakoplakia diagnosis through specific stain technology. -
Key words:
- malakoplakia /
- Michaelis-Gutmann bodies /
- specific stain
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图 1 光镜下软斑病的同心圆状排列的M-G小体（H & E×400）
Figure 1. Concentric-layered M-G bodies in malakoplakia observed bylight microscope(H & E×400)
图 2 过碘酸-Schiff染色法检测软斑病中的M-G小体（PAS染色×400）
Figure 2. Periodic acid-Schiff reaction showed the M-G bodies in malakoplakia(pas×400)
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