【摘要】：目的:提高對(duì)軟斑病(malakoplakia)的發(fā)病機(jī)制、臨床病理學(xué)特征的認(rèn)識(shí)及診療水平,避免誤診、漏診。方法:收集山西省腫瘤醫(yī)院2007年3月至2017年3月診斷為軟斑病4例患者的臨床資料,觀察其組織病理學(xué)形態(tài),行免疫組織化學(xué)及特殊染色。4例患者平均發(fā)病年齡為63(56~76)歲。其中男性2例、女性2例,發(fā)生于膀胱2例、雙側(cè)輸尿管1例、右側(cè)盆腔1例。1例有糖尿病史患者合并系統(tǒng)性紅斑狼瘡,另3例無特殊疾病史。結(jié)果:4例患者的病變組織學(xué)形態(tài)均顯示大量片狀分布的組織細(xì)胞、同心圓狀排列的嗜堿性Michaelis-Gutmann(M-G)小體、淋巴細(xì)胞、漿細(xì)胞、嗜酸性粒細(xì)胞及中性粒細(xì)胞浸潤。免疫組織化學(xué)法顯示組織細(xì)胞PGM-1及CD68陽性。鈣、鐵染色及PAS染色中M-G小體均陽性。結(jié)論:軟斑病是一種罕見肉芽腫性病變,可在多個(gè)器官形成瘤樣結(jié)節(jié)。多累及泌尿生殖系統(tǒng),但在身體各器官均可發(fā)生。無特異性的臨床表現(xiàn),影像學(xué)多表現(xiàn)為占位性病變,因此極易被臨床誤診為惡性病變。確診主要依靠病理診斷,因其罕見性致使病理工作者易漏診,其中特殊染色方法對(duì)診斷具有較大的幫助。
[Abstract]:Objective: to improve the understanding of pathogenesis, clinicopathological features and diagnosis and treatment of soft spot disease (malakoplakia) in order to avoid misdiagnosis and missed diagnosis. Methods: the clinical data of 4 patients diagnosed as soft spot from March 2007 to March 2017 in Shanxi Cancer Hospital were collected and their histopathological features were observed. The average age of onset was 63 (56 / 76) years. Among them, 2 cases were male, 2 cases were female, 2 cases occurred in bladder, 1 case was bilateral ureter, 1 case was right pelvis, 1 case had diabetic history with systemic lupus erythematosus (SLE), and the other 3 cases had no history of special diseases. Results: all the 4 patients showed a large number of patchy tissue cells, concentric round arrangement of basophilic Michaelis-Gutmann bodies, lymphocytes, plasmacytes, eosinophils and neutrophils. Immunohistochemical staining showed that PGM-1 and CD68 were positive in tissue cells. M + G bodies were positive in calcium, iron and PAS staining. Conclusion: soft spot is a rare granulomatous lesion that can form tumor-like nodules in multiple organs. Multiple involvement in the genitourinary system, but can occur in all organs of the body. It is easy to be misdiagnosed as malignant lesion because of its non-specific clinical manifestation and space-occupying lesions. The diagnosis mainly depends on pathological diagnosis. Because of its rarity, pathologists are easy to miss diagnosis, among which the special staining method is of great help in diagnosis.
【作者單位】： 山西省腫瘤醫(yī)院病理科;
【分類號(hào)】：R593.9

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1 馬鴻達(dá);張乃鑫;;軟斑病二例報(bào)告[J];天津醫(yī)藥;1990年05期

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