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目的探讨膀胱器官相关性假肉瘤性肌纤维母细胞性增生(PMP)临床病理特征、诊断与鉴别诊断、治疗及预后。方法对3例膀胱PMP的临床表现、组织形态学及免疫组化进行观察并结合文献分析。结果该病临床主要表现为无痛性肉眼血尿,有尿频、尿急、尿痛或排尿困难。组织形态上,病变多位于黏膜下,梭形细胞显著增生,排列紊乱,穿插于正常的膀胱肌层内,间质内常伴有较多的炎细胞浸润,嗜酸性粒细胞易见;梭形细胞胞质丰富,核呈圆形、卵圆形或胖梭形,可见显著核仁,核分裂象罕见,间质可见黏液样变性。免疫组化:梭形细胞a-SMA(3/3)、calponin(3/3)、desmin(1/3)和h-caldesmon(1/3)(+),其中1例少数梭形细胞ALK和CK-pan(+);3例CD34、Dog-1、S-100、β-catenin、myogenin和MyoD1均(-)。结论膀胱器官相关性假肉瘤性肌纤维母细胞性增生是一种发生于膀胱的假肉瘤样良性病变,因其呈假性浸润生长模式,细胞有一定异型,有时易误诊为恶性软组织肉瘤,需要结合免疫组化鉴别。
Objective To investigate the clinical and pathological features, diagnosis, differential diagnosis, treatment and prognosis of cystic organ-associated pseudo sarcoma myogenic dysplasia (PMP). Methods The clinical manifestations, histomorphology and immunohistochemistry of 3 cases of bladder PMP were observed and analyzed by literature. Results The clinical manifestations of the disease mainly painless gross hematuria, frequent urination, urgency, dysuria or dysuria. Morphology, the lesions are located in the submucosa, spindle cells significantly hyperplasia, arranged disorder, interspersed within the normal bladder muscle layer, the interstitial often accompanied by more inflammatory cell infiltration, eosinophil easy to see; spindle Cytoplasm is rich in cells, the nucleus was round, oval or fat fusiform, visible nucleoli, mitotic figures rare, interstitial mucus-like degeneration. Immunohistochemistry: a-SMA (3/3), calponin (3/3), desmin (1/3) and h-caldesmon (1/3) And CK-pan (+); 3 cases of CD34, Dog-1, S-100, β-catenin, myogenin and MyoD1 (-). Conclusions Bladder organ-associated pseudo sarcoma myofibroblastic hyperplasia is a pseudosarcoma-like benign lesion in the bladder. Because of its pseudo-invasive growth pattern, the cells have a certain shape, sometimes misdiagnosed as malignant soft tissue sarcoma, and need to be combined Immunohistochemical identification.