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目的探讨胸膜弥漫性黏液性恶性纤维组织细胞瘤(MFH)的临床和病理特点。方法分析我院收治的1例胸膜弥漫性黏液性 MFH 患者的临床和病理资料,并分析国内外文献报道的7例胸膜 MFH 病例。结果患者为男性,65岁,表现为咳嗽、胸痛、气促伴大量血性胸腔积液。胸部 CT示右侧脏壁层胸膜弥漫性肿块影。开胸探查见脏壁层胸膜表面弥漫性灰白色半透明肿块,直径为1～8 cm。光镜下可见黏液性基质中瘤细胞弥漫分布,呈梭形和多形性。波形蛋白、CD_(68)、溶菌酶免疫组化染色瘤细胞呈阳性反应,细胞角蛋白、上皮膜抗原、结蛋白呈阴性反应。术后24 d,因上腔静脉梗阻、循环衰竭死亡。与文献报道的非弥漫性胸膜 MFH 相比,本例为胸膜弥漫性多灶性 MFH,起病急,病情进展快。结论胸膜弥漫性黏液性 MFH 是非常罕见的胸膜原发性肿瘤,恶性程度高,预后极差。
Objective To investigate the clinical and pathological features of pleural diffuse mucinous malignant fibrous histiocytoma (MFH). Methods The clinical and pathological data of one case of pleural diffuse mucinous MFH admitted to our hospital were analyzed. Seven pleural MFH cases reported in the literature were analyzed. Results The patients were male, 65 years old, manifested as cough, chest pain, shortness of breath with massive bloody pleural effusion. Chest CT showed the right parietal pleura diffuse mass shadow. Thoracotomy to explore the visceral pleural surface diffuse gray translucent mass, a diameter of 1 ~ 8 cm. Light microscopic tumor cells found in mucinous matrix diffuse distribution, fusiform and pleomorphic. The vimentin, CD_ (68), lysozyme immunohistochemical staining of tumor cells were positive, cytokeratin, epithelial membrane antigen, desmin was negative. At 24 days after operation, the patients died of circulatory failure due to superior vena cava obstruction. Compared with the non-diffuse pleural MFH reported in the literature, pleural diffuse multifocal MFH, acute onset, rapid progression. Conclusion Pleural diffuse mucinous MFH is a very rare primary pleural tumor with a high degree of malignancy and a very poor prognosis.